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Outcomes

Rheumatic and Immunologic Diseases


2006
Outcomes | 2006

Quality counts when referring patients to hospitals

and physicians, so Cleveland Clinic has created a series

of outcomes books similar to this one for its institutes

and departments. Designed for a health care provider

audience, the outcomes books contain a summary of

our surgical and medical trends and approaches; data

on patient volume and outcomes; and a review of new

technologies and innovations. We hope you find these

data valuable. To view all our outcomes books, visit

Cleveland Clinic’s Quality Web site at

clevelandclinic.org/quality/outcomes.
 | Rheumatic and Immunologic Diseases 2006
Rheumatic and Immunologic Diseases | 

Table of Contents |

Chairman’s Letter 5

Department Overview 6

Center for Osteoporosis & Metabolic Bone Diseases 10

Center for Vasculitis Care and Research 12

Recent Awards and Appointments 14

Selected Studies 15

Quality & Outcome Measures 20

Patient Experience 45

Innovations 46

New Knowledge 50

Staff Listing 56

Department Contacts | How to Refer Patients 60

Locations 61

Cleveland Clinic Overview 62

Online Services 63

Cleveland Clinic Contact Numbers 64


 | Rheumatic and Immunologic Diseases 2006
Rheumatic and Immunologic Diseases | 

Chairman’s Letter |

Cleveland Clinic’s Department of Rheumatic and Immunologic Diseases has a


long-standing commitment to excellence in patient care and training physicians
for future generations. The highest quality care depends on progress in clinical
trials, outcomes research and understanding illness at genetic, molecular and
cellular levels. Both clinical and basic research hold promises of discovering new
treatment strategies and cures for our patients.

The department has grown to include individuals with expertise in all areas of
clinical rheumatology. Recruitment has emphasized selection of faculty with
complementary skills in clinical, educational and research arenas. We have had
the good fortune of growing in an environment that is rich in opportunities for
collaboration in areas related to our own work in immunology, metabolic bone
disease, orthopaedics, cardiovascular medicine and surgery, pathology and
imaging. We have benefited from research partnerships with valued colleagues
around the world. Opportunities for discovery are unprecedented and are likely
to bear fruit, in large part because of team efforts that ignore intellectual and
geographic boundaries.

In the following pages, you will meet with members of our department and
specialty teams, and consider how we at the Clinic can best serve you and your
patients.

Gary S. Hoffman, M.D., M.S.


Chairman, Department of Rheumatic and Immunologic Diseases
 | Rheumatic and Immunologic Diseases 2006

Department Overview |
A high volume of patients allowed the department to accomplish its primary goal
to provide care for the sick. It also allowed us to meet our complementary goals in
research and education.

Main Campus New Patient Visits


1,500

1,000

500

0
2002 2003 2004 2005 2006

Total new patient visits for Main Campus and Family Health
Centers for 2006 was 1,797.

Main Campus Total Vists


25,000

20,000

15,000
#
10,000

5,000

0
2002 2003 2004 2005 2006

Total visits for Main Campus and Family Health Centers for
2006 was 41,934.
Rheumatic and Immunologic Diseases | 

Top Primary Diagnoses

The breadth of skills within the Department of Rheumatic and Immunologic


Diseases attracts patients who have a wide range of illnesses, both common
and rare.
2002 2003 2004 2005 2006

Osteoporosis 504 566 709 1,077 1,569

Rheumatoid Arthritis 1,306 1,215 1,280 1,251 1,565

Fibromyalgia 1,402 1,193 1,229 1,241 1,354

Systemic Lupus Erythematosus 446 402 472 483 562

Bursitis 256 265 288 204 518

Wegener’s 242 253 264 329 365

Osteoarthritis 247 188 216 373 345

Gout 155 137 185 232 333

Psoriatic Arthritis 212 161 178 175 253

Arteritis NOS / Vasculitis 145 185 179 179 250

Scleroderma 134 117 168 213 241

Juvenile Rheumatoid Arthritis 242 195 216 214 218

Giant Cell Arteritis 81 83 128 108 110

Ankylosing Spondylitis 60 49 58 64 103

Polymyositis 80 78 77 85 101

Dermatomyositis 90 81 88 92 96

Takayasu’s Disease 53 58 81 81 78

Hypersensitivity Angiitis 34 33 33 42 43

Cerebral Arteritis 48 49 38 37 39

Bechet’s Disease 24 25 35 39 33

Polyarteritis Nodosa 48 30 22 36 27

Reiter’s Syndrome 19 11 26 22 26

Pseudogout 36 19 11 24 16

Henoch Schoenlein Purpura 11 11 7 10 12


 | Rheumatic and Immunologic Diseases 2006

Grant support from non-commercial sponsors continues to grow. This has been
most successful in the areas of vasculitis, metabolic bone disease and outcomes
research.

Active Research Grants


14
Federal and Foundation Grants
Commercial

# 7

0
2002 2003 2004 2005 2006
Rheumatic and Immunologic Diseases | 

Total Publications
100

# 50

0
2002 2003 2004 2005 2006
10 | Rheumatic and Immunologic Diseases 2006

Center for Osteoporosis and Metabolic Bone Diseases

Chad Deal, M.D.

The Center for Osteoporosis and Metabolic Bone Diseases is a multidisciplinary clinic
staffed by rheumatologists, endocrinologists and radiologists.

In addition, the Center has strong ties with the Departments of Biomedical Engineering,
Orthopaedic Surgery and the Orthopaedic Research Center where molecular
mechanisms of bone formation, skeletal repair and bone growth are active areas of
basic and clinical research using cell-based therapies.

Steady growth in faculty and patient numbers occurred in the Center for Osteoporosis
and Metabolic Bone Disease.

Main Campus Osteoporosis


3,000
Unique Patient
Total Patient Visits

2,000

1,000

0
2002 2003 2004 2005 2006
Rheumatic and Immunologic Diseases | 11

Main Campus and Regional Medical Practice


Osteoporosis
12,000
Unique Patient
Total Patient Visits

8,000

#
4,000

0
2006

Below is a graph demonstrating growth in Bone Densitometry since the opening of


the Center for Osteoporosis and Metabolic Bone Diseases.

Bone Densitometry
20,000

# 10,000

0
2002 2003 2004 2005 2006
12 | Rheumatic and Immunologic Diseases 2006

Center for Vasculitis Care and Research

Carol Langford, M.D.

Unique Vasculitis Patients

The Center for Vasculitis Care and Research is a major area of service, research and
educational commitment.

New Patient Vasculitis Volume*


1,000

750

# 500

250

0
2002 2003 2004 2005 2006

Outpatient Vasculitis Cases*


4,000

3,000

# 2,000

1,000

0
2002 2003 2004 2005 2006

* Main campus only. Data does not include Family Health Centers.
Rheumatic and Immunologic Diseases | 13

Some of the most commonly encountered vasculitis diagnoses are presented in


the table:

Unique Forms
of Vasculitis 2002 2003 2004 2005 2006

Wegener’s 242 253 264 329 365

Arteritis NOS / Vasculitis 145 185 179 179 250

Giant Cell Arteritis 81 83 128 108 110

Takayasu’s Disease 53 58 81 81 78

Hypersensitivity Angiitis 34 33 33 42 43

Cerebral Arteritis 48 49 38 37 39

Bechet’s Disease 24 25 35 39 33

Polyarteritis Nodosa 48 30 22 22 27

Henoch Schonlein Purpura 11 11 7 10 12

Total Vasculitis Cases 686 727 787 847 957


14 | Rheumatic and Immunologic Diseases 2006

Recent awards and appointments: Highlights of 2006

Dr. Abby Abelson


Three-year Clinical Scholar Educator Award from the American College of Rheumatology

Dr. Leonard Calabrese


Board of Directors of the Research and Education Foundation for the American College
of Rheumatology | Deputy Editor Arthritis Care and Research | American College of
Rheumatology Delegate to the Decade of the Bone and Joint World Health Organization.

Dr. Chad Deal, Chair, Advisory Group on US Rheumatology Workforce |


Investigator-initiated trial of zolendronic acid therapy following Forteo for osteoporosis
(Novartis). The trial is designed to evaluate the effectiveness of an intravenous
bisphosphonate, zolendronic acid, after patients finish a course of the anabolic agent for
osteoporosis, Forteo.

Dr. Gary Hoffman


Reappointment to the FDA Advisory Panel for arthritis drugs
Appointment as Editor of Current Opinion in Rheumatology
Sam and Maria Miller Award for Excellence in Clinical Research

Dr. Elaine Husni


Two-year Clinical Investigator Award from the American College of Rheumatology
Research and Education fund. This award supports investigation of atherosclerosis in
rheumatoid arthritis. Executive committee member of the PRECISION trial, worldwide
multicenter trial of over 20,000 patients to assess the cardiovascular risk profile of
three commonly used nonsteroidal anti-inflammatory medications (celecoxib, ibuprofen
and naproxen) in patients with osteoarthritis and rheumatoid arthritis.

Dr. Carol Langford


Pilot project grant, Rare Diseases Clinical Research Network (RDCRN), National Institutes
of Health | Mechanistic studies of T cell regulation in Wegener’s granulomatosis | Co-
editors: Fauci AS and Langford CA. Harrison’s Rheumatology. New York: McGraw Hill;
2006.

Dr. Brian Mandell


Editor ACP Medical Knowledge Self Assessment Program 14. Rheumatology book.
2006. Chairman of the American College of Rheumatology National Meeting Planning
Committee | American College of Physicians annual meeting planning committee
(member) | Editor of the Merck Manual (Rheumatology and Immunology sections)
Rheumatic and Immunologic Diseases | 15

Selected Studies and Why They Are Important

Stone JH, Holbrook JT, Marriott MA, Tibbs AK, Sejismundo LP, Min Y-I, Specks U,
Merkel PA, Spiera R, Davis JC, St. Clair EW, McCune WJ, Ytterberg SR, Allen NB,
Hoffman GS. Solid malignancies among patients in the Wegener’s granulomatosis
etanercept trial. Arthritis Rheum 2006; 54:1608-1618.
This study demonstrated the concurrent use of etanercept and cyclophosphamide
is associated with an increased risk of solid tumors in patients with Wegener’s
granulomatosis. The combined use of these agents should be avoided.

Villa-Forte A, Clark TM, Mascha E, Karafa MT, Gomes M, Carey JJ, Arrigain S,
Roberson G, Hoffman GS. Wegener granulomatosis: Customized treatment using
cyclophosphamide and methotrexate. A 12-year single-practice experience. Arthritis
Rheum 54 (S): 1178. 2006.
This study demonstrated minimizing cyclophosphamide use or, when possible,
avoiding it in patients with mild disease is associated with excellent outcomes and
survivals in patients with Wegener’s granulomatosis.

Finkielman JD, Merkel PA, Schroeder D, Hoffman GS, Spiera R, St. Clair EW, Specks
U for the WGET Research Group. Antineutrophil cytoplasmic antibodies against
proteinase 3 do not predict disease relapses in Wegener’s granulomatosis. Arthritis
Rheum 2006; 54: s835-836.
This study demonstrated that changes in ANCA titers correlate poorly with disease
activity and risk of relapse in Wegener’s granulomatosis and should not be used as a
guide to therapy.

Koening CL, Langford CA, Kirchner HL, Hoffman GS. Renal graft survival in Wegener’s
granulomatosis (WG): Comparison to systemic lupus erythematosus (SLE) from a
national database. Arthritis Rheum 2006; 54:s486.
This study demonstrated that patients with Wegener’s granulomatosis who require
renal transplantation have excellent outcomes and only rarely have recurrent disease
within the graft.

Molloy ES, Langford CA, Clark TM, Gota CE, Calabrese LH, Hoffman GS. Durable
remission in patients with refractory Takayasu’s arteritis treated with infliximab and
etanercept. Arthritis Rheum 2006; 54:s487.
This study addressed the difficulty in achieving lasting remissions for patients
with Takayasu’s arteritis. The experimental use of infliximab led to unprecedented
improvement in most patients who had previously been treatment-resistant.
16 | Rheumatic and Immunologic Diseases 2006

Lee MS, Smith SD, Galor A, Hoffman GS. Antiplatelet and anticoagulant therapy in
patients with giant cell arteritis. Arthritis Rheum 2006;54:3306-3309.
This study demonstrated the use of low-dose aspirin could reduce cerebral ischemic
events in giant cell arteritis by a factor of 3, without producing serious side effects.

Hoffman GS, Cid MC, Rendt KE, Merkel PA, Weyand CM, Stone JH, Salvarani C, Xu
W, Visvanathan S, Rahman MU for the Infliximab-GCA study group. Prednisone and
infliximab for giant cell arteritis: a randomized, double-blind, placebo-controlled,
multicenter study of efficacy and safety. Ann Intern Med May 2007; 146(a):621-30.
This study was the first-ever randomized double-blind trial of a biologic agent in GCA.
It demonstrated the use of infliximab in GCA did not add value to usual therapy with
corticosteroids.

Molloy E, Calabrese LH. Tumor-like mass lesions: an under-recognized presentation of


primary angiitis of the central nervous system. Arthritis & Rheum 2006; 54(9):S486.
This series highlights the potential for CNS vasculitis to present as mass lesions and
indicates when this presentation should be considered.

Bharadwaj SS, Hajj-Ali RA, Hammel JP, Calabrese LH. Evolution of the syndrome of
benign angiopathy of the central nervous system (BACNS): retrospective review of 48
patients. Arthritis & Rheum 2006; 54(9):S485.
This study represents an important review of the clinical features, diagnoses and
treatments of BACNS.

Hajj-Ali RA, Yee A, Calabrese LH. Central nervous system vasculitis secondary to
sarcoidosis. Arthritis & Rheum 2006; 54(9):S492.
This report describes the clinical features seen when sarcoidosis manifests as a CNS
vasculitis.

Deal C, Barr W, Harrington T, Hooker R, Hogan P, Bouchery E, Birnbaum N for the ACR
Workforce Subcommittee COTW. US Rheumatologist Supply and Demand: 2005-2006
Workforce Study. Arthritis Rheum 2007; 56(3):722-729.
This study developed a model to predict rheumatology supply and demand through
2025.

Solomon DH, Chibnik LB, Losina E, Huang J, Fossel AH, Husni ME, Katz JN.
Development of a preliminary index that predicts adverse events after total knee
replacement. Arthritis Rheum 2006;54:1536-42.
This paper examined patient and hospital level factors that may be important to predict
poor outcomes following total knee replacement surgery.
Rheumatic and Immunologic Diseases | 17

Harrington T, Deal C. Successes and failures in improving osteoporosis care after


fragility fracture: results of a multi-site clinical improvement project. Arthritis Rheum
(Arthritis Care & Research). 2006;55:724-728.
Demonstration of a practice improvement project to address the critical issue of
evaluation and treatment after hip fracture.

Calabrese LH, Zein N, Vassilopoulos D. Hepatitis B (HBV) reactivation with


immunosuppressive therapy in rheumatic diseases. Ann Rheum Dis 2006; 65:983-
989.
This review represents an important dialogue in the rheumatology profession for
examining and exploring the problem and proposing guidelines for avoiding serious
and/or fatal cases of HBV reactivation.

Oliveira S, Ravelli A, Pistorio A, Castell E, Malattia C, Prieur AM, Saad-Magalhaes C,


Murray KJ, Sang-Cheol B, Joos R, Foeldvari I, Duarte C, Wulffraat N, Lahdenne P,
Dolezalova P, de Inocencio J, Pratsidou-Gertsi P, Hofer M, Nikishina I, Ozgogan H,
Hashkes P, Martini A, Ruperto N for the Pediatric Rheumatology International Trials
Organization (PRINTO). Health related quality of life of patients with juvenile idiopathic
arthritis: The PRINTO multinational quality of life cohort study. Arthritis Rheum. In
press. This very large international study included 6,667 subjects (3,352 with juvenile
idiopathic arthritis (JIA) and 3,315 controls) showed a significant impairment of health-
related quality of life among JIA patients, especially in the physical domains in all types
of JIA except persistent oligoarthritis.

Uziel Y, Butbul-Aviel Y, Barash J, Padeh S, Mukamel M, Gorodnitski N, Brik R, Hashkes


PJ. Recurrent transient synovitis of the hip in childhood: the long-term outcome among
39 patients. J Rheumatol 2006;33:810-811.
This is the first paper to study the long-term outcome of this very common form of
episodic arthritis in children.

Husni, ME, Bershadsky B, Worley S, Barsoum W, Muschler G. Clinical variation in


health status and outcome following primary hip and knee arthroplasty. American
Academy of Orthopedic Surgeons. 73rd Annual Meeting Proceedings Chicago, IL.
2006; 7:128.
This study examines preoperative clinical factors that may be important to predict
outcomes following total knee and hip arthroplasty.
18 | Rheumatic and Immunologic Diseases 2006

Muschler GF, Husni ME, Lograsso M, Barsoum W, Thornton J, Worley S,


Bershadsky B. Early outcome assessment comparison of unilateral and bilateral
primary total knee arthroplasty. American Association of Orthopedic Society. 2006.
American Academy of Orthopedic Surgeons. 73rd Annual Meeting Proceedings.
Chicago, IL. 2006; 7:132.
This study examines early outcomes of unilateral versus bilateral total knee
arthroplasty in a large academic setting.

Muschler GF, Rossi S, Lograsso M, Husni E, Gerz D, Emmerich C, Thornton J, Worley


S. A practical quality reporting system for hip and knee arthroplasty procedures.
American Association of Orthopedic Society. 2006. American Academy of Orthopedic
Surgeons. 73rd Annual Meeting Proceedings. Chicago, IL. 2006; 7:287.
This study assesses the use of an innovative patient self report system following total
knee and hip arthroplasty.

Qureshi AA, Cohen D, Mody E, Husni ME. Development and evaluation of PASE: A
self-administered psoriatic arthritis screening and evaluation tool. J Am Acad Dermatol.
2007. In press.
Given emerging therapies for psoriatic arthritis, this abstract demonstrates an
innovative, patient self-report tool to help screen and evaluate for psoriatic arthritis.

Mody, E Qureshi AA, Husni, ME. Diagnosis of arthritis in psoriasis patients presenting
with joint pain to a dermatology-rheumatology clinic. Br J of Dermatol. 2007. In press
This study highlights the importance of proper diagnosis of musculoskeletal complaints
in patients with psoriasis and psoriatic arthritis. In patients with psoriasis, not all joint
pain is consistent with psoriatic arthritis.

Whyte M, Mumm S, Deal C. Adult Hypophosphatasia Treated with Teriparatide. J Clini


Endocrinol Metab 2007; 92:1203-1208.
This is the first study to document effective treatment for hypophosphatasia.
Rheumatic and Immunologic Diseases | 19

Carey JJ, Delaney MF, Richmond B, Cromer BA, Love TE, Lewis S, Thomas CA,
Miller PD, Licata AA. Equivalence of technology generated T-scores and Z-scores in
young adult bone densitometry. J Bone Miner Res 2006; 21: S351.
This study showed that DXA-generated T-scores and Z-scores in young adults may
differ significantly and substantially.

Schulte ME, Licata AA, Carey JJ, Delaney MF. Inappropriate PTH response to severe
vitamin D deficiency in patients with chronic liver disease. J Bone Miner Res 2006;
21: S435.
This study showed people with liver disease and vitamin D deficiency have lower
than expected corresponding PTH levels.

Carey JJ, Delaney MF, Cromer BA, Love TE, Lewis S, Thomas CA, Miller PD, Licata
AA, Richmond B. Diagnostic agreement between DXA generated T-scores and Z-
scores in young adults. International Bone Densitometry Workshop, Kyoto, Japan.
November 2006. Oral presentation.
This study shows use of DXA-generated Z-scores instead of T-scores in young adults
results in significant diagnostic discordance using either 1994 W.H.O. or 2005
I.S.C.D. criteria.

Richmond B, Delaney MF, Cromer BA, Love TE, Lewis S, Thomas CA, Miller PD,
Licata AA, Carey JJ. The impact of body weight on DXA generated Z-scores in young
adults. International Bone Densitometry Workshop, Kyoto, Japan. November 2005.
Oral presentation.
This study shows adjustment for body weight accounts for a large part of the
differences seen between DXA-generated Z-scores and T-scores and that this
adjustment is inappropriate.

Sikon AL, Thacker HL, Carey JJ, Deal C, Licata AA. Secondary Osteoporosis: Are We
Recognizing It? J Women’s Health. 2006; 15:1174-83.
This study demonstrates secondary causes of low bone mass should be screened
as they are very common in people with low Z-scores.
20 | Rheumatic and Immunologic Diseases 2006

Quality & Outcomes Measures |


Arthritis Clinical Research

Elaine Husni, M.D., M.P.H.

Prospective Randomized Evaluation of Celecoxib Integrated Safety Versus


Ibuprofen or Naproxen (PRECISION)

Systemic rheumatic diseases affect approximately 20 million Americans, over 5%


of the population. In connective tissue diseases, such as rheumatoid arthritis and
systemic lupus erythematosus (SLE), there is a marked increase in cardiovascular
(CV) complications compared with patients without a systemic rheumatic disease. In
addition to CV risk, common treatments used for these diseases, non-steroidal anti-
inflammatory drugs (NSAIDs) especially cyclooxygenase (COX)-2 inhibitors, may also
increase a patient’s CV risk.

What is the study purpose?


This clinical trial will evaluate the overall benefit and risk of celecoxib, a Cox 2
inhibitor, compared to two commonly prescribed traditional (non-selective) non-
steroidal anti-inflammatory drugs (NSAIDs) in the treatment of arthritis pain. The trial
definitively addresses the relative safety of celecoxib compared with ibuprofen and
naproxen. While rofecoxib was found to significantly increase CV events, the evidence
is less clear with celecoxib. Observational studies reported less CV risk with celecoxib
compared with rofecoxib, but similar risks compared with naproxen or ibuprofen. This
trial is funded by Pfizer, but will be conducted at Cleveland Clinic under the leadership
of Dr. Steven E. Nissen; Dr. Elaine Husni will be the principal investigator.

What is the study design?


Approximately 20,000 patients with osteoarthritis (OA) or rheumatoid arthritis (RA) on
a chronic analgesic regimen for >6 months, with existing CV disease or at high risk
for disease, will be randomized to celecoxib, ibuprofen or naproxen in this double-
blind, triple-dummy, multicenter, multinational study utilizing a 3-arm parallel group
design. All patients will receive esomeprazole for gastro protection. Aspirin will be
administered for cardiovascular prophylaxis when clinically indicated.
Rheumatic and Immunologic Diseases | 21

Who qualifies for this study?


Adult patients with a clinical diagnosis of OA or RA for at least 6 months and who
have required a chronic analgesic regimen for at least 6 months and patients with
established or who are at high risk for CV disease qualify for this study.

Every 6 months

Celecoxib 100-200 mg twice daily

Established or at high
risk for CVD
Ibuprofen 600-800 mg three times daily

Diagnosis of symptomatic Screen Naproxen 375-500 mg twice daily


osteoarthritis or
rheumatoid arthritis

-3wks Rand M1 M2 M3 M4 M8 M12 M24 M30 M36 M42 M48

Visit 1 2 3 4 5 6 7 8 9 10 11 12 13

Minimum follow-up

What is the primary endpoint for this study?


The primary composite endpoint is the first occurrence of cardiovascular death,
non-fatal MI or non-fatal stroke. This is an event-driven trial. Patients will be followed
a minimum of 18 months until 762 events have occurred.

The PRECISION trial is the first study to enroll patients with existing CV disease
(or high risk of disease) undergoing treatment with a COX-2 inhibitor compared
with nonselective non-steroidal anti-inflammatory agents. This large scale study
will define the CV safety profile of celecoxib versus ibuprofen and naproxen
and, thereby, help determine the optimal strategy for pain control in this at-risk
population.
22 | Rheumatic and Immunologic Diseases 2006

Development and Evaluation of PASE: A Self-administered Psoriatic


Arthritis Screening and Evaluation Tool

M. Elaine Husni, M.D., M.P.H. and Abrar Qureshi, M.D., M.P.H.

Psoriatic arthritis may be an under-recognized disorder. Nearly one of three


patients with psoriasis alone may develop psoriatic arthritis and, often, these
patients are solely followed by dermatology providers. Complications associated
with psoriatic arthritis can be prevented with early diagnosis and referral to a
specialist. Our objective was to evaluate the reliability and validity of the PASE
questionnaire to detect inflammatory arthritis in patients with psoriasis.

What is being studied?


We developed a self-administered instrument (the Psoriatic Arthritis Screening and
Evaluation questionnaire – PASE) that allows dermatologists to screen psoriasis
patients for signs and symptoms of inflammatory arthritis. A multidisciplinary
team of experts was involved in the design of the questionnaire. PASE was
developed using standardized methodology for the development of both functional
and health-related instruments geared toward musculoskeletal diseases. This
study provided the means to evaluate the reliability and validity of PASE for
detecting an inflammatory arthritis.

How was this studied?


Patients with psoriasis-like skin complaints who presented to a combined
dermatology/rheumatology clinic at an academic medical center were studied
from October 2005 to September 2006 and stratified according to the clinical
diagnosis made by a Board-certified dermatologist and rheumatologist. All
patients completed a PASE questionnaire of 15 five-choice questions divided
into two subscales: symptoms and function. Diagnosis of psoriasis or psoriatic
arthritis was based on clinical evaluation, history and physical, and radiographs,
if necessary, by a Board-certified rheumatologist and dermatologist. The goal of
the analyses was to evaluate the ability of the PASE total, function and symptom
scores, to distinguish psoriatic arthritis from non-psoriatic arthritis patients.
Rheumatic and Immunologic Diseases | 23

What were the study results?


A total of 108 psoriasis patients completed PASE questionnaires. Thirty-seven
patients were excluded due to missing data or inadequate responses. Of 71
participants, 18 were diagnosed with psoriatic arthritis and 53 with psoriasis
alone. The PASE questionnaire distinguished psoriatic arthritis from psoriasis with
specificity of 74% and sensitivity of 83%. The Wilcoxon rank sum test was used
to test for differences between these groups. Receiver Operator Curves (ROC)
was used to choose the best cut-point for each score by optimizing sensitivity
and specificity in predicting psoriatic arthritis. In addition, 95% exact binomial
confidence intervals were calculated. Both the subscale scales scores were
significantly different between the psoriatic arthritis and non-psoriatic arthritis
groups. Cronbach’s alpha coefficient had good internal consistency (raw 0.937
and standardized 0.938).

What does this mean in clinical practice?


In patients with psoriasis, the PASE questionnaire is a reliable and valid tool to
help screen patients for psoriatic arthritis. There is a need to identify patients
with psoriatic arthritis so that earlier referral to a rheumatologist can be made,
timely therapy can be initiated and long-term disability avoided.

Total PASE Score


8
7
6
5
# 4
3
2
1
0
Psoriasis Psoriatic Arthritis

Scatter plot of total PASE scores for participants with psoriasis and psoriatic
arthritis. The horizontal line represents a score of 47 which, in this study, best
differentiates the psoriatic arthritis (PsA) group from the non-PsA group.
24 | Rheumatic and Immunologic Diseases 2006

Self Assessed Health Status and Outcome Following Primary Hip and
Knee Arthroplasty

Elaine Husni, M.D. M.P.H., Wael Barsoum, M.D., George F. Muschler, M.D., and
Boris Bershadsky, Ph.D.

Total joint arthroplasty is generally considered an effective procedure, but the


current literature does not support specific recommendations about which
patients are most likely to benefit from this surgery. Previous studies focused on
baseline functional status, preoperative pain levels and prosthesis survival. Less
attention was been placed on patient reported outcomes which may have the
potential of being modifiable prior to surgery.

What is being studied?


The study is to determine if patient self-assessment of health (including mental
health and psychosocial variables) and presence of comorbidities are associated
with outcome for patients undergoing primary total hip and knee replacement
surgery.

How did we study this?


A prospective arthroplasty registry that provided demographics, co-morbidities
and SF-36 data before and one-year after surgery was studied. Between
December 2000 and March 2004, 835 patients underwent primary total knee
arthroplasty (TKA) and 1,008 underwent primary total hip arthroplasty (THA). A
total of 16 joint arthroplasty surgeons performed the procedures. Data were
gathered on preoperative and postoperative information provided by 489 patients
(232 TKA and 257 THA). The relationship between functional outcomes 1-year
postoperatively with patient characteristics, including mental health, associated
comorbidities and patient demographics, was assessed.
Rheumatic and Immunologic Diseases | 25

What were the results?


Preoperative SF 36 scores (physical component score (PCS), mental component
score (MCS), age, and number of co-morbid conditions) were significant
predictors of the magnitude of improvement in PCS one year following surgery.
Greater improvement of PCS was associated with better MCS, younger age, less
co-morbid conditions while higher body mass index (BMI) was associated with
worse PCS. In addition, greater BMI was associated with lower preoperative PCS.
Preoperative SF 36 scores (PCS and MCS) were significant predictors of the
magnitude of improvement in their MCS score at one year following surgery.

How does this improve patient care?


Our results suggest an association between preoperative mental health and BMI
with outcome measured by improvement in PCS in patients undergoing hip and
knee arthroplasty. This suggests a role of preoperative intervention targeting
patients with higher BMI and worse mental component scores in order to improve
outcomes. Reasons that may explain the association of poor mental health
preoperatively and worse functional status include reduced activity levels, lack
of motivation to overcome functional impairments and inability to adopt healthier
lifestyles. This supports current literature findings for the importance of targeting
this patient population with increased education and emotional support prior to
total knee and hip replacement surgery.

Risk factors and associated interventions to improve outcomes of patients


undergoing total knee and hip arthroplasty.

Potential
interventions

Maintain healthy
pre op weight

Preoperative counseling

More frequent
post op counseling
Risk factors
for poor outcome

High BMI

Low mental score

SF-36
26 | Rheumatic and Immunologic Diseases 2006

Patients Undergoing Total Hip (THA) Versus Total Knee Arthroplasty (TKA):
Does One Procedure Have a Better Outcome?

Elaine Husni, M.D. M.P.H., Wael Barsoum, M.D., George F. Muschler, M.D., and
Boris Bershadsky, Ph.D.

Orthopedic outcomes for total knee arthroplasty and total hip arthroplasty are well
described in the orthopedic literature. Little published data, however, compares
the health outcomes of each procedure. Conflicting reviews exist on which type
of arthroplasty procedure, hip or knee, provides greater overall improvement.
Current literature suggests patients who undergo total hip replacements improve
more than patients who undergo total knee replacements.

What is being studied?


To address gaps in the literature, a large sample of patients who underwent
TKA and THA was studied. One-year postoperative outcomes were analyzed
using the most comprehensive model. We propose that measuring SF-36
preoperatively and one-year postoperatively may be a practical resource to
identify characteristics of the cohort that may lead to better outcomes.

How did we study this?


The primary objective of this study was to prospectively compare THA versus
total TKA with respect to improvement in general health-related quality-of-life
(HRQoL) outcomes.

What were the results?


The improvement in postoperative PCS for hip and knee replacement is highly
dependent on comprehensive review of baseline characteristics of the patient
cohort. In examining THA and TKA, improvement in PCS is associated with lower
age, lower BMI and lower number of comorbidities. Better MCS at baseline is
significantly associated with better improvement in the physical component.
Rheumatic and Immunologic Diseases | 27

How does this improve patient care?


It is critically important to include health-related quality-of-life variables (socio-
demographic data, body mass index, comorbidities and social/emotional status)
to help isolate characteristics associated with improved outcomes for each
procedure. The comparisons between THA and TKA can better allow patients and
surgeons to understand the relative risks and benefits of the procedures when
similar criteria are used.

Risk adjustment should be included as a mandatory component for orthopedic


observational studies. The non adjusted comparison of observations studies has
potential for selection bias. Future research should be aimed at developing a
minimal core set of risk adjustors needed to demonstrate baseline equivalency
of comparing two groups. This will help clinicians make more informed decisions
regarding which types of patients will benefit most from which particular joint
replacement surgery.

Interpretation of DXA Generated T-scores and Z-scores


Carey JJ, Delaney MF, Love TE, Cromer BA, Richmond BJ, Miller PD, Manilla-McIntosh M,
Thomas CL, Lewis SA, Licata AA. The Cleveland Clinic, Case Western Reserve University
and The Colorado Center for Bone Research.

Background
Bone density (DXA) measures bone density in grams per centimeter2 (g/cm2).
Using this measurement, DXA software calculates a T-score and Z-score. Clinical
guidelines for the management of osteoporosis incorporate T-scores rather than
actual bone density in g/cm2. T-scores are not used in premenopausal women
or men under age 50 where only Z-scores are reported. For these patients,
low bone mass for age is diagnosed at a Z-score <-2.0 and a workup for
secondary osteoporosis is usually recommended. Both T-scores and Z-scores are
dependent on several factors: bone density measurement, skeletal site, subject
demographics, DXA manufacturer and DXA software used. Alterations in any of
these factors could lead to changes in the T-score or Z-score value.
28 | Rheumatic and Immunologic Diseases 2006

Conceptually, T-scores and Z-scores should be identical or very similar in young adults.
At the Center for Osteoporosis and Metabolic Bone Disease, however, surprisingly
large differences in some young individuals have been noticed. We undertook a
comprehensive study of this phenomenon in order to better understand the magnitude
of these differences and their implications for clinical practice and osteoporosis guidelines.

Methods
A retrospective cohort was used to evaluate the equivalence of DXA generated T-scores
and Z-scores in young adults scanned on either Hologic or Lunar machines. Multiple
analyses were performed to describe and compare differences between measures.

Results
Young adult Z-scores and T-scores differed substantially and significantly in men and
women for both technologies. These differences resulted in significant diagnostic
discordance if Z-scores were substituted for T-scores, using either 1994 World Health
Organization or 2005 International Society for Clinical densitometry diagnostic criteria.
The folded empirical cumulative distribution plot of the differences between the T-score
and Z-score at the total hip site for one technology is shown.

50

40

30
%
20

10

0
-2.0 -1.5 -1.0 -0.5 0.0 0.5 1.0 1.5
hip z-score - hip t-score

Conclusions and Significance


Large differences may be seen between T-scores and Z-scores in young adults.
Substitution of Z-scores for T-scores may result in significant ascertainment bias.
DXA-generated Z-scores should be interpreted with caution in clinical practice.
Standardization of Z-score definition and method of calculation is needed to avoid
mistakes in both diagnoses and evaluation recommendations.
Rheumatic and Immunologic Diseases | 29

Transplant Evaluation: Center for Osteoporosis and Metabolic Bone


Disease

Miriam Delaney, M.D., Chad Deal, M.D., Abby Abelson, M.D., Angelo Licata, M.D.,
Ph.D., John Carey, M.D.

Cleveland Clinic has one of the largest organ transplant programs in the United
States. In 2006, there were 594 transplants performed. These patients are at
risk for osteoporosis because of underlying disease (liver disease with vitamin
D deficiency or alcohol use) or lung disease (steroid treatment of chronic
obstructive pulmonary disease (COPD), low body weight and malabsorption with
cystic fibrosis), or bone marrow transplant (graft versus host disease and steroid
use). All transplant recipients receive steroids at the time of transplant and,
often, for long periods of time after surgery. Fracture rate in this population is
significant.

In 2004, the Center for Metabolic Bone Disease established a transplant


evaluation program in coordination with the transplant center. The purpose was
to evaluate clinically and obtain a bone density and treat patients at the time of
transplant evaluation or soon after transplantation. The percent of liver transplant
patients having a bone center evaluation increased from 22% in 2004 to 43%
in 2005 to 69.2% in 2006. A similar program is ongoing for cardiac and lung
transplant patients.

Liver Transplant Patients with Osteoporosis Clinic Evaluations


80

60

% 40

20

0
2004 2005 2006
30 | Rheumatic and Immunologic Diseases 2006

R.J. Fasenmyer Center for Clinical Immunology

Leonard Calabrese, D.O.

Toward Better Diagnosis and Treatment of Vasculitis of the Central Nervous


System (CNS)

Central nervous system vasculitis is one of the rarest and most challenging forms
of vascular inflammatory diseases. For nearly 20 years we have been interested in
and working in this area, attempting to clarify subsets of patients with more benign
outcomes who may be candidates for less aggressive therapies. While reversible
vasoconstriction has long been appreciated in the wake of subarachnoid hemorrhage, it
was only suspected as playing a role in CNS inflammatory diseases in the early 1990s.
A disorder labeled Benign Angiopathy of the Central Nervous System (BACNS) was first
described by our group in 1993 and believed to be, but not proven, a form of reversible
cerebral vasoconstriction. These patients were mostly labeled as true CNS vasculitis
based upon angiographic appearance, but they appeared to have a far more benign
course.

Over the past decade, more of these patients have been evaluated and subjected
to repeated neurovascular imaging within a few weeks to months, demonstrating
remarkable and rapid reversal of vascular abnormalities. These patients are now
designated as having Reversible Cerebral Vasoconstrictive Syndromes (RCVS). These
can occur idiopathically or can be seen in a variety of clinical settings: 1) after head
trauma of neurovascular surgical procedure 2) in the postpartum period and 3)
associated with an increasing number of drugs including sympathomimetics such as
ephedrine, cocaine and others. Diagnostic features of the syndrome are noted in the
following table.
Rheumatic and Immunologic Diseases | 31

Characteristics Number of Cases Percentage


Total n=48
Mean Age 43.8 years+ 11.6 years
Male age 34.1 + 5.0 years
Female age 44.9 + 11.6 years
Females 43/48 89.6
Race
Caucasian 37/48 77.1
African American 5/48 10.4
Others 6/48 12.5
Associated Triggers
Postpartum state 2/48 4.1
Sympathomimmetic amines 4/48 8.3
Social marijuana 7/48 14.6
Smoking history 20/48 41.7
PMHx Migraine 11/48 22.9
Clinical Signs/Symptoms
Headache (HA) 43/48 89.6
Classic thunderclap HA 18/48 37.5
Thunderclap like 14/48 29.1
Migraine HA 6/48 12.5
Coitus HA 7/48 14.5
Exercise HA 4/48 8.3
Valsalva HA 8/48 16.6
Recurrent Headaches 20/48 41.6
Neurological s/s 7/48 14.6
Focal 3/48 6.2
Diffuse 3/48 6.2
Both 1/48 2.0
Seizures 2/48 4.2
Transient ischemic attack 3/48 6.3
Isolated visual symptoms 10/48 20.8
without stroke (blurry, diplopia,
transient loss, floaters, etc.)
Sought medical care on day of 18/48 37.5
Symptom onset
32 | Rheumatic and Immunologic Diseases 2006

Summary of Critical Elements for Diagnosis of RCVS

1. Transfemoral angiography or indirect (CT or MR) angiography documenting


segmental cerebral artery vasoconstriction.

2. No evidence for aneurysmal subarachnoid hemorrhage.

3. Normal or near-normal cerebrospinal fluid analysis (proteins of <60 mg%,


white blood cells < 10 per mm3, normal glucose).

4. Severe, acute headache, with or without additional neurologic signs or


symptoms.

5. The diagnosis cannot be confirmed until reversibility of the angiographic


abnormalities is documented with 12 weeks after onset, or if death occurs
before the follow-up studies are completed. Autopsy rules out conditions
such as vasculitis, intracranial atherosclerosis and aneurysmal subarachnoid
hemorrhage, which can also manifest with headache and stroke.

In 2006, the largest series of such patients was presented at the American
College of Rheumatology meeting in Washington, DC. A summary of these
patients is presented in the table. Of note is the fact that angiographic follow-up
was available in 38 patients, showing reversibility in 97%.
Rheumatic and Immunologic Diseases | 33

Optimal treatment for this disorder has not yet been established, as opposed to
patients with true vasculitis of the central nervous system who require prolonged
therapy with glucocorticoids and cytotoxic drugs. RCVS patients can often be
treated with observation or calcium channel blockers alone.

This is a cerebral angiography of a 40 y/o female who presented with 3 weeks


of severe headaches and an episode of transient paresis of her left arm. An MRI
was normal and CSF studies were normal. Her angiogram revealed multiple areas
of stenosis and dilatation in multiple vessels (left) which resolved one month after
treatment with prednisone 60 mg/day and verapamil 240 mg/day (right). RCVS
was diagnosed.

The outcome was favorable in the majority with persistent morbidity related to
whether or not the patient experienced stroke at presentation.

Outcome
Total recovery 23/48
Recovery with residual neurological deficits 16/48
Death 0/48
Relapse 3/48
Not known/lost to follow-up 6/48

In 2007, working with colleagues at the Massachusetts General Hospital and


the Mayo Clinic, a definitive review of this disorder was published in the Annals
of Internal Medicine which, for the first time, integrates the clinical features and
pathophysiologic concepts into a single review for a broad medical audience.

Hajj-Ali R, Furlan A, Abou-Chebel A, Calabrese LH: Benign angiopathy of the central nervous system
(BACNS): Cohort of 16 patients with clinical course and long-term follow-up. Arthritis Care and Research
2002;47:662-669.

Calabrese LH, Gragg LH, Furlan AJ: Benign angiography: A distinct subset of angiographically defined
primary angiitis of the central nervous system. J Rheumatology 1993;20:2046-2050.

Calabrese LH, Dodick DW, Schwendt T, Singhal A: Narrative review: reversible cerebral vasoconstrictive
syndromes. Ann Int Med 2007;146:34-44.
34 | Rheumatic and Immunologic Diseases 2006

Center for Vasculitis Care and Research

Carol Langford, M.D.

Renal Graft Survival in Wegener’s Granulomatosis (WG): Comparison to


Systemic Lupus Erythematosus (SLE) from a National Database

Curry L. Koening, Carol A. Langford, H.L. Kirchner, Gary S. Hoffman

Nearly three-fourths of Wegener’s granulomatosis (WG) patients will develop


glomerulonephritis at some time during their disease course. Despite treatment
with immunosuppressive drugs, up to 20% of patients develop end-stage renal
disease (ESRD). For patients with ESRD, kidney transplantation is an important
medical advance. Although case reports and small cohort studies report
excellent graft survival rates in WG, it is not known how these results compare to
patients who undergo renal transplant for more common rheumatic conditions.
This led us to compare renal graft survival rates in patients transplanted for WG
to patients transplanted for systemic lupus erythematosus (SLE). The study used
data from the Organ Procurement and Transplantation Network’s (OPTN) national
transplant database. Renal graft survival rates were compared among patients
who received a first episode renal transplantation for either WG or SLE between
October 1987 and February 2006. A total of 982 patients received renal
transplantation for WG and 6,574 patients for SLE.

100

75

% 50
Survival

25
WG
Log-Rank P < 0.0001
SLE
0
0 200 400 600 800 1,000
Weeks to Failure
Rheumatic and Immunologic Diseases | 35

Wegener’s Granulomatosis Systemic Lupus Erythematosus


Rate (95% CI) Rate (95% CI)

Survival Rate

6 month 95.6% (94.1-96.7) 92.7% (92.1-93.3)

1 year 95.2% (93.6-96.4) 90.7% (90.0-91.4)

5 year 90.0% (87.9-91.7) 79.7% (78.7-80.7)

Kaplan Meier analysis of the two groups showed patients transplanted for WG had
better graft survival rates at 6 months, 1 year, and 5 years compared to patients
transplanted for SLE. After adjusting for various confounding factors, patients
transplanted for SLE had a 71% (P<0.0001 95% CI 43%- 104%) increased risk
of renal graft failure compared to patients with WG. Furthermore, the number of
patients reported to have recurrent disease as a cause for their graft failure was
small in both WG and SLE patients (4.3% and 4.8% respectively).

This is the largest study to compare renal graft survival rates in patients
transplanted for WG to those transplanted for SLE. Results show patients
transplanted for WG had better graft survival rates than those transplanted for
SLE. How graft survival rates in WG patients compare to those transplanted for
more common, non-rheumatic causes of renal failure will be determined in the
future from this database.
36 | Rheumatic and Immunologic Diseases 2006

Substitution of Methotrexate for Cyclophosphamide in Wegener’s


Granulomatosis

A 12-year Single-practice Experience

Alexandra Villa-Forte, Tiffany M. Clark, Marcelo Gomes, John Carey, Edward


Mascha, Matthew T. Karafa, Susana Arrigain, Gerald Roberson and Gary S.
Hoffman

Objective
The objective of the study is to assess outcomes of therapy in newly-diagnosed
Wegener’s granulomatosis (WG) using methotrexate (MTX) for mild-to-moderate
disease or short-term treatment with cyclophosphamide followed by MTX for
severe disease.

Methods
Retrospective review: Patients with WG were included if their initial plan of therapy
and subsequent care were directly supervised by the Cleveland Clinic Center for
Vasculitis Care and Research. Severe disease (i.e. immediately life-threatening
or involving critical organs) was initially treated with cyclophosphamide and
glucocorticoids. Mild-to-moderate disease was initially treated with MTX and
glucocorticoids if serum creatinine was less than 2mg/dl. Following initial
improvement of severe disease, treatment was changed to MTX if serum
creatinine was originally less than 2mg/dl or had diminished to less than 2mg/dl.
Disease activity was determined at each visit and later converted to a Birmingham
Vasculitis Activity Score, modified for Wegener’s granulomatosis (BVAS/WG).
Laboratory monitoring of disease and treatment toxicity was initially weekly and
never less often than monthly.

Results
Eighty-two (32%) of 253 patients referred to the Center for Vasculitis Care and
Research with WG met eligibility criteria. Ineligible patients did not have new
onset disease or were not able to be followed principally in our center. Seventy
percent of patients (57/82) initially had severe disease and received a short
course of cyclophosphamide for remission-induction. In over half of these
patients, illness was judged to be severe because of pulmonary hemorrhage,
rapidly progressive glomerulonephritis, including need for dialysis, or neurologic
abnormalities.
Rheumatic and Immunologic Diseases | 37

All patients improved; remission was achieved in 50% (41/82) of patients within
6 months and 72% (59/82) within 12 months. Sustained remission (BVAS/WG
= 0, for at least 6 consecutive months) was ultimately achieved in 77% (63/82)
of patients. Among patients with sustained remissions, the mean duration of
remission prior to relapse was 20.5 months (SD ± 21.2).

Time to Remission
100

75

% 50

25
BVAS/WG of zero

0
0 1 3 3
Years (follow-up)

Seventy-five patients (91%) achieved remissions of any duration [mean duration


± SD = 11.2 ± 16.8 months]. Among the 75 (91%) patients who achieved
remission of any duration, 45% relapsed within 1 year and 66% relapsed within
2 years after remission. Eighty-two percent of relapsed patients achieved
subsequent remissions after additional treatment. About three-fourths of relapses
were mild and promptly responded to treatment.

Seventeen percent of patients developed serious infections. Cyclophosphamide-


associated cystitis or bladder cancer did not occur in any patients. At least one
form of permanent morbidity from WG alone was noted in 74% of patients. Three
patients (3.7%) died over a median follow-up period of 4.5 years. No deaths were
due to active disease.

Although treatment was primarily directed toward achieving clinical improvement


and not calculated to achieve marked lymphopenia, patients in whom treatment
produced lymphocyte counts of less than < 500/mm3, sustained over a median
time of 4 (quartiles, 1-8.5) months, were 3.8 times more likely to achieve a
sustained remission (> 6 months; p = 0.015). Conversely, following remission,
an absolute lymphocyte count of >1000/mm3 was associated with a hazard ratio
for relapse of 3.0, although the latter difference was not statistically significant.
38 | Rheumatic and Immunologic Diseases 2006

Conclusions
In patients with Wegener’s granulomatosis, a strategy that limits or avoids
cyclophosphamide therapy has produced excellent results as judged by frequency
of remissions, survival and avoidance of long-term cyclophosphamide toxicity.
Relapses, however, were common and incremental permanent morbidity occurred
in most patients. While not a goal of therapy, when treatment produced marked
lymphopenia, prolonged remissions were more likely.

Evolution of the Syndrome of Benign Angiopathy of the Central Nervous


System (BACNS)

Retrospective review of 48 patients

Swati S. Bharadwaj, Rula A. Hajj-Ali, Jeffrey P. Hammel, Leonard H. Calabrese

Purpose
The entity of BACNS was first proposed in 1993 as a subset of Primary Angiitis
of Central Nervous System (PACNS), defined by acute headache and/or stroke,
benign cerebrospinal fluid, high probability cerebral angiogram and a favorable
clinical outcome without intense immunosuppression. In 2002, we described a
series of 16 patients with BACNS with rapid reversal of angiographic changes
suggesting the underlying mechanism was reversible vasoconstriction rather than
true vasculitis. Recognizing this entity is clinically important to spare patients
from aggressive studies such as a brain biopsy and the toxicities of unnecessary
immunosuppression. The clinical description of BACNS was extended with a
larger cohort of 48 patients in an attempt to refine our diagnostic and treatment
strategies.

Methods
A retrospective chart review was conducted on BACNS patients. Diagnosis was
based on prior descriptions (Hajj-Ali Arth Rheum 2002; 47:662-669). Information
was collected on demographics, triggers, strokes, headaches, visual symptoms,
seizures, laboratory markers, spinal taps, brain biopsies, initial and follow-up
neuroimaging, treatments, outcomes and dates. Information was entered into an
Oracle Database and a descriptive analysis conducted.
Rheumatic and Immunologic Diseases | 39

Results
Forty-eight patients were included in the study. Mean age was 43.8 years;
90% were females. Associated factors were marijuana use in 14.6% and
sympathomimetic amine use in 8.3%. Past history of migraine was seen in
22.9%. Headache was most common and present in 89.6%, stroke in 14.6%,
isolated visual symptoms in 20.8% and seizure in 4.2%. Of the 83.3% of patients
who had spinal taps, 82.5% had normal WBCs and 85% had normal CSF proteins
(7.5% were not reliable due to trauma/bleed, 7.5% had mild elevations). Brain
biopsy was performed in 22.9% and found normal in all except one patient
with a concomitant glioma. Initial MRI was normal in 17.1%. Subsequent MRIs
revealed abnormalities in 81.4% (44.2% ischemia, 18.6% cerebral hemorrhage,
14% subarachnoid hemorrhage). Stroke was bilateral or multiple in 53%. All
patients had high probability neurovascular imaging abnormalities consistent with
vasculitis. Follow-up neurovascular imaging performed in 79.2% demonstrated
reversibility of the vascular abnormality in all except one recent patient with
a premature MRA. Median time to reversibility was 92 days. Treatment was
non-standardized with brief course glucocorticoids used most frequently; more
recently, patients were encountered receiving calcium channel blockers. Total
clinical recovery occurred in 47.9%; recovery with residual deficits occurred in
33.3%; relapses occurred in 3%.

Conclusions
BACNS is a common mimic of PACNS and appears to represent a syndrome
of reversible cerebral vasoconstriction and not true cerebral vasculitis. Initial
neuroimaging can be negative in many patients. Outcome morbidity appears to
be limited to stroke. Studies to define validated diagnostic criteria and optimal
therapy are needed.
40 | Rheumatic and Immunologic Diseases 2006

Takayasu’s Arteritis: Guarded Outcomes in an American Cohort

Kathleen Maksimowicz-McKinnon, Tiffany M. Clark, Gary S. Hoffman

Purpose
1) To describe clinical, laboratory, and radiographic manifestations of Takayasu’s
arteritis (TAK) in an American cohort. 2) To evaluate response to interventions,
remission and relapse rates and disease progression. 3) Compare observations
to cohorts from the United States, Japan, India, Italy and Mexico.

Methods
Seventy-five patients were retrospectively studied using a uniform database that
included clinical, laboratory and imaging data. Vascular imaging studies were
performed at least yearly to monitor disease progression.

Results
Ninety-two percent of patients were Caucasian; 89% female. Median age at onset
was 26 years; median duration of follow-up was 3 years. Common manifestations
included loss or asymmetry of pulse (57%), limb blood pressure discrepancy
(53%) and bruits (53%). Eleven percent of patients were asymptomatic prior to
disease diagnosis. Angiographic studies demonstrated aortic abnormalities in
79% of patients. Subclavian (65%) and carotid (43%) arteries were also frequently
affected.

Ninety-three percent of longitudinally followed patients attained disease remission


of any duration, but only 28% attained a sustained remission of at least 6 months
duration while receiving less than 10 mg/day of prednisone. Both angioplasty
and vascular surgery procedures were initially successful, but recurrent stenosis
occurred in 78% of angioplasty and 36% of bypass/reconstruction procedures.
Vascular claudication limited routine daily activities in 60%. Cerebral ischemic
disease occurred in 17%. Complete occupational disability occurred in 23%
which correlated with number of relapses. There were two disease-related
deaths.

Disease manifestations in our cohort are similar to the NIH, Italian, Japanese and
Mexican cohorts in female predominance and disease manifestations, but differed
from the Indian cohort in that the latter group had a higher frequency of males,
abdominal aortic involvement and hypertension.
Rheumatic and Immunologic Diseases | 41

Cohort Comparisons: Demographics And Diagnostic Evaluations

Cohort Patient Mean Age Caucasian Female Vascular PTA/surgical Mortality (%)
(#) at onset (%) (%) imaging (%) intervention (%)

CCF 5 26 92 89 100 48 3

NIH (3) 60 25 * 75 97 100 50 3

Italy (6) 104 40 99 88 100 50 NR

India (7) 106 27 NR 61 90** 13 11

Japan (8)

(Ueda) 52 24 NR 81 90 ** NR 12 ***

Japan (9)

(Nakao) 84 NR NR 86 64 NR 7

Japan (10)

(Ishikawa) 120 30 * 0 93 NR ** 12 13

Mexico (11)

(Lupi-Herrera) 107 NR NR 84 100 21 14

NR = not reported. *Only median age reported. **Diagnosis and disease features were assessed at least once in the
entire cohort by vascular imaging, at the time of surgery, or at autopsy. ***Based on reported number of patients that
underwent autopsy.

Manifestations at disease onset in the American,


Italian, and Indian cohorts.*

Aortic arch
Thoracic aorta
Abdominal aorta
*
Left carotid
Right carotid
Left subclavian
Right subclavian
Celiac *
* CC
Superior mesenteric * NIH
*
Left renal Italy
Japan
Right renal
India
Left iliac Mexico
*
Right iliac
*

0 25 50 75 100
% Affected

*Instances in which data were not provided for a cohort are indicated by absence of a comparison bar.
42 | Rheumatic and Immunologic Diseases 2006

Vascular Interventions and Outcomes in the Longitudinal Cohort*

Aneurysm/dilatation Site Bypass/reconstruction (#) Patent (%)**


Aortic root 7 86
Descending aorta 1 100
Axillary 1 100

Stenosis Site Bypass (#) Patent (%) Angioplasty (#) Patent (%)
Abdominal aorta NA NA 1 100
Carotid 7 71 2 0
Axillary 3 0 2 100
Subclavian 5 80 4 0
Coronary 12 50 1 100
Mesenteric 2 100 NA NA
Renal 5 60 8 0
Iliofemoral 1 100 ***
2 100 ***

* 21 of 30 patients in longitudinal cohort required revascularization procedures.


**Sustained over the period of observation, without further intervention required.
***1 patient with iliofemoral bypass and one with iliofemoral angioplasty had partial
restenosis at follow-up imaging.

Conclusions
Takayasu’s arteritis in the United States is characterized by chronic, relapsing disease
in the majority of patients. Although most patients are able to attain remission with
the use of glucocorticosteroid therapy, attaining sustained steroid-free remission
is uncommon. The majority of disease relapses in our cohort occurred while
patients were on immunosuppressive therapy. Seventy percent of patients required
revascularization procedures.

In contrast to prior studies from Indian cohorts, sustained vascular patency following
angioplasty was infrequent. Sustained vascular patency at 3 years was superior for
arterial bypass/reconstruction (68%) compared with angioplasty (25%). Although
mortality rates were low, chronic morbidity and disability were frequent in this young
population. The frequency of disease relapse and the need for revascularization
highlight the inadequacy of current therapy for Takayasu’s arteritis.
Rheumatic and Immunologic Diseases | 43

Durable Remission in Patients with Refractory Takayasu’s Arteritis Treated


with Infliximab or Etanercept

Eamonn S. Molloy, Carol A. Langford, Tiffany M. Clark, Carmen E. Gota, Leonard


H. Calabrese, Gary S. Hoffman

While up to 20% of Takayasu’s arteritis (TAK) patients have a monophasic


illness, most have a relapsing/remitting course, typically requiring prolonged
treatment with glucocorticoids and additional immunosuppressive agents, such
as methotrexate, azathioprine and, in severe cases, cyclophosphamide. More
than one-third of patients are unable to achieve adequate disease control on
these therapies. As tumor necrosis factor alpha (TNFa) is critical for granuloma
homeostasis and granulomatous inflammation is the pathologic hallmark of
TAK, TNFa is an attractive therapeutic target in TAK; anti-TNF therapy has been
employed in TAK patients with encouraging results.

We sought to assess the efficacy of anti-TNF therapy in patients with TAK


refractory to other therapies. Twenty-eight TAK patients were identified as having
received anti-TNF therapy; 3 patients were excluded because of insufficient follow-
up data. No patient was in remission at the time of initiation of anti-TNF therapy;
13 had not achieved remission at any time since initial diagnosis. Twenty-five
patients were treated with anti-TNF therapy for up to 6.5 years. Twenty patients
were treated with infliximab (INF) with a mean follow-up of 27 months (range 2-76).
Nine patients were treated with etanercept (ETA) with a mean follow-up of 33
months (range 4-78). Four patients received both agents (all initially treated with
ETA, later switched to INF). No patients were treated with adalimumab.

Of 25 patients, 22 were female; mean age was 35 years (range 15-63). Patients’
ethnicities were Caucasian, 20, Asian,3, Hispanic,1, and one unknown. Median
disease duration was 108 months (range 31-336). All patients were previously
treated with prednisone and a mean of 2 additional immunosuppressive agents
(range 0-6) including 22 methotrexate, 10 cyclophosphamide, and 12 with a
variety of other agents.
44 | Rheumatic and Immunologic Diseases 2006

Major Outcomes in Refractory TAK Patients Treated with


anti-TNF Therapy.
Anti-TNF Agent Infliximab Etanercept
Patients treated 20 9
Complete remission* 12 4
Partial remission** 5 2
Discontinuation within 6 months 3 3
Treatment failure 1 3
- Adverse event 1 -
- Other 1 -

*Complete remission defined as resolution of symptoms, lack of new changes


on vascular imaging and discontinuation of glucocortocoids for at least 6 months.
**Partial remission defined as resolution/improvement in symptoms, lack of new
changes on vascular imaging and tapering of glucocortocoids to <10mg/day of
prednisone or equivalent, for at least 6 months.

Overall, prednisone was discontinued in 15/25 patients (60%) and tapered below
10 mg/day in a further 7/25 (28%). Median prednisone dose before and after
anti-TNF therapy was 19 mg (range 5-90) and 0 mg (range 0-30). Nine out of 18
patients were able to taper or discontinue additional immunosuppressive agents
used concurrently with the anti-TNF agent. Six patients had definite new changes
noted on MR imaging (3 INF, 3 ETA); 5 patients entered remission on higher dose
anti-TNF therapy. Three patients who stopped ETA had disease flares within
a median of 2 months (range 1-2); 6/7 patients that stopped INF had disease
flare at a median interval of 6 months (range 2-12); 1 patient recommenced INF
treatment and achieved sustained remission.

Conclusions
In this group of TAK patients refractory to other immunosuppressive therapies,
anti-TNF therapy led to complete or partial remission in 79% of patients. Anti-
TNF therapy allowed prednisone to be discontinued or tapered in 60% and 28%,
respectively. Of those patients taking concurrent immunosuppressive drugs other
than prednisone, anti-TNF therapy allowed reduction of the dose of these agents
in 50%. These findings provide further support for the rationale for randomized
controlled trials of anti-TNF therapy in TAK.
Rheumatic and Immunologic Diseases | 45

Patient Experience |

We ask our patients about their experiences and satisfaction with the services
provided by our staff. Although our patients are already indicating we provide
excellent care, we are committed to continuous improvement.

Outpatient
Overall Rating of Care 2006
100 100

80 80

60 60
%
40 40

20 20

0 0
Excellent Very Good Good Fair Poor
46 | Rheumatic and Immunologic Diseases 2006

Innovations |
The Future of the Rheumatology Workforce in the United States 2005-2025.

Authors: Chad Deal, M.D., Chair, Subcommittee on Workforce, Roderick Hooker, Ph.D.,
P.A., Timothy Harrington, M.D., Neal Birnbaum, M.D., Paul Hogan, Ellen Bouchery,
Marisa Klein-Gitelman, Walter Barr, M.D.

The American College of Rheumatology (ACR) retained the Lewin Group to conduct a
workforce study in 2005-2006. The purpose was to develop and apply a model that
allows prediction of current and future supply and demand for rheumatology services in
the United States. A supply model was developed using the age and sex distribution of
current physicians, retirement and mortality rates, the number of fellowship slots and fill
rates, and practice patterns of rheumatologists. A survey instrument was designed and
sent to 1,683 U.S. rheumatologists to obtain more detailed information, including work
effort, productivity, measures of excess demand, and retirement plans. A computer-
based Markov projection model was created to project supply and permit sensitivity
analyses of factors affecting the future workforce. Supply and demand was assumed
to be equal in 2005, although there is likely to be current excess demand for adult and
pediatric services. (The computer model can be adjusted to reflect different scenarios.)

The number of adult rheumatologists in 2005 was 4,946. Male and female
rheumatologists are equally distributed up to age 44, though above age 44, males
are more predominate. By 2025, the percent of women in the adult rheumatology
workforce is projected to increase from 30.2% to 43.6% and in the pediatric workforce,
from 51.5% to 62.7%. The mean number of patient visits is 3,758 for male and 2,800
for female rheumatologists. The productivity of rheumatologists under age 40 was
lower than older rheumatologists. If the supply and demand in 2025 were assumed
to be equal, the demand for rheumatologists is projected to exceed supply by 2,576
adult (supply=4,643, demand=7,219) and 33 pediatric (supply=254, demand=287)
rheumatologists. (Current data suggest the pediatric rheumatology workforce is
experiencing a substantial excess of demand versus supply.)

Primary factors in the excess demand include: an aging population (which will increase
the number of people with rheumatic disorders), growth in the real per capita income
and flat rheumatology supply due to fixed numbers entering the workforce and retiring.
Rheumatic and Immunologic Diseases | 47

Unknown affects that could influence these projections include technology advances,
practice redesign, changes in insurance reimbursement and shifting lifestyles. Based
on assessment of supply and demand under current scenarios, the demand for
rheumatologists is expected to exceed supply in the coming decades. Strategies
for the profession to adapt to this changing healthcare landscape include: increasing
the number of fellows (an additional 188 slots would be required), utilizing physician
assistants and nurse practitioners in greater numbers and improving practice
efficiency.

The R.J. Fasenmyer Center for Clinical Immunology is dedicated to


patient care, education and research in clinical immunology with an emphasis on
autoimmunity and chronic viral illness. As part of our goal to support education,
the construction of a state-of-the art meeting center within the Department of
Rheumatic and Immunologic Diseases will occur in 2008. This center will expand
the educational mission of the Fasenmyer Center and the Department of Rheumatic
and Immunologic Diseases to students, residents and physicians. A comprehensive
immunology curriculum has been developed with ongoing lectures in immunology
and autoimmunity.

Julie C. Huang, Stanley L. Hazen, Gary Hoffman, Elaine Husni.

Abstract
Impact of a Best Practice Clinical Alert on Preventive Cardiology Referral
for Patients with Systemic Rheumatic Disease

Julie C. Huang, Stanley L. Hazen, Gary Hoffman, Elaine Husni.

There is known increased risk of cardiovascular morbidity and mortality in patients


with systemic rheumatic diseases. Despite significant cardiovascular risk, there is
low utilization of preventive cardiology services at our institution.

Purpose
To assess the impact of a best practice clinical alert on referral patterns of patients
with systemic rheumatic diseases from the Cleveland Clinic Department of Rheumatic
and Immunologic Diseases to the Section of Preventive Cardiology.
48 | Rheumatic and Immunologic Diseases 2006

Methods
An automatic clinician alert system was implemented via the electronic medical record
in June 2006. Any patient with a diagnosis of systemic rheumatic disease seen in
the Cleveland Clinic Department of Rheumatic and Immunologic Diseases triggered a
“pop-up” alert recommending referral to Preventive Cardiology for cardiovascular risk
assessment. The Section of Preventive Cardiology PreCIS® database was then queried
using SAS data retrieval for a monthly report of patients referred from Rheumatology
or for a diagnosis of systemic rheumatic disease. The referral pattern was compared
to that prior to implementation of the best practice clinical alert to assess its impact on
referrals to Preventive Cardiology.

Results
In the three months following implementation of the best practice clinical alert system,
the monthly average referral rate increased by 1,495%. The majority of patients
required some form of cardiac risk intervention, including initiation of statin therapy for
dyslipidemia, electrocardiogram, and/or non invasive cardiac testing for suspicion of
coronary artery disease.

Conclusion
Use of a best practice clinician alert system is a highly effective means of increasing
referral of patients with systemic rheumatic diseases to preventive cardiology.
Addressing cardiovascular risk in this high risk patient population is critical. Further
research will be needed to determine whether adoption of best practice clinical alerts
results in reduced cardiovascular burden in this group of patients.

Education
Abby Abelson, M.D., Education Program Director of the Department of Rheumatic
and Immunologic Diseases, was awarded the Clinical Scholar Educator Grant of the
American College of Rheumatology Research and Education Foundation for July 2007-
2010.

Decisions about career choices are often made during medical school and early in
resident training. Medical school curricula include rheumatology sections in the first two
years. Clinical rheumatology faculty teach medical students and residents during clinical
Rheumatic and Immunologic Diseases | 49

rotations. We feel a well-designed Web-based curriculum that would be available to all


rheumatology faculty could improve students’, residents’ and fellows’ understanding of
the field of rheumatology and provide more informed decisions on career choices.

Dr. Abelson’s grant provides support to develop an interactive Web-based, case-based


rheumatology curriculum that will facilitate the teaching of the clinical and basic science
of rheumatic disease to medical students, residents and rheumatology fellows. The
format will be case-based, problem-based learning (PBL) curriculum and will increase
medical students’ and residents’ knowledge of the rapidly evolving field of rheumatic
diseases. Cleveland Clinic College of Medicine at Case Western University has
established expertise in Web- and case-based medical education that will be utilized for
curriculum development. The diversity of the disease mechanisms, illnesses, prognoses
and large numbers of new effective therapies will be addressed in this curriculum. The
flexible Web-based format will allow the curriculum to be utilized in a variety of settings,
from individual self-directed learning to small conference or larger group seminar
settings, therefore maximizing the opportunities for medical educators to expose
students and residents to the depth and breadth of rheumatology.

Best Practices: Steroid Alert in Epic


Steroids are frequently used to treat many diseases across all medical specialties.
Current American College of Rheumatology guidelines recommend a bone density in
patients on more than 5 mg of prednisone for more than three months. In addition,
treatment with a bisphosphonate is recommended in patients at initiation of steroid
therapy and in patients with ongoing steroids at T-score < -1.0.

To improve compliance with ACR guidelines, a best practice alert system was initiated
in the Department of Rheumatic and Immunologic Diseases and for all rheumatologists
in the Cleveland Clinic system. Epicare evaluates the patient’s medical record for steroid
use of more than three months, whether a bone density has been done in the last 12
months and an alert screen is generated with a smart form that allows the clinician to
order a bone density test if indicated.
50 | Rheumatic and Immunologic Diseases 2006

New Knowledge |
Selected Publication Highlights

Calabrese LH. Primary Angiitis of the Central Nervous System. In Rheumatology. 4th
ed. Hochberg M, Silman A, Smolen J, Weinblatt M, Weisman MH, eds. St. Louis, Mo.:
Mosby; 2006.

Vassilopoulos D, Calabrese LH. Rheumatic aspects of human immunodeficiency virus


infection and other immunodeficiency states. In Rheumatology. 4th ed. Hochberg M,
Silman A, Smolen J, Weinblatt M, Weisman MH, eds St. Louis, Mo.: Mosby; 2006.

Fauci AS, Langford CA, eds. Harrison’s Rheumatology. New York: McGraw Hill; 2006.

Hoffman GS, Langford CA, Calabrese LH. In Vasculitis Hospital Medicine 2nd ed.
Wachter RM, Goldman L, Hollander H, eds. Philadelphia, Pa: Lippincott, Williams and
Wilkins; 2005: 1121-33.

Stone JH, Hoffman GS. In Rheumatology 4th ed. Hochberg MC, Silman AJ, Smolen JS,
Weinblatt ME, Weisman MH, eds. Wegener’s granulomatosis. St. Louis, Mo.: Mosby;
2007. In press. Chapter in book

Villa-Forte A, Santos A, Hoffman GS. In Head and Neck Manifestations of Systemic


Diseases. Harris J, Weisman M, eds. Vasculitis and Otolaryngology; New York, Marcel
Dekker; 2007.

Hajj-Ali R, Mandell BF. Rajagopalan, Mukherjee, eds. Approach to and Management


of Inflammatory Vascular Disease in Manual of Vascular Diseases. Philadelphia, Pa:
Lipincott, Williams and Wilkins; 2005.
Rheumatic and Immunologic Diseases | 51

Mandell BF, Johnson B. The patient with arthritis or systemic autoimmune disease.
In Just the Facts in Perioperative Medicine, Cohn, Smetana, Weed, eds. New York.:
McGraw Hill; 2006.

Mandell BF. Differential diagnosis of arthritis. American College of Physicians Medical


Knowledge Self Assessment Project. 14. Rheumatology. 2006.

Mandell BF. Septic Arthritis. American College of Physicians Medical Knowledge Self
Assessment Project. 14. Rheumatology. 2006.

Mandell BF. Crystal Disease. American College of Physicians Medical Knowledge Self
Assessment Project. 14. Rheumatology. 2006.

Mandell BF. Perioperative management of the patient with rheumatologic disease. In:
Medical Consultation of the Surgical Patient. Merli, Weitz eds Philadelphia, Pa: Lipincott
Press; In press 2007.

Mandell BF. Dale D, ed. Systemic Vasculitis. ACP Scientific American Medicine. 2006.

Deal C, Abelson A, Carey J. Management of Osteoporosis. In: Rheumatology. 4th ed.


Hochberg MC, Silman AJ, Smolen JS, Weinblatt ME, Weisman MH, eds. St Louis, MO:
Mosby; In press 2007.
52 | Rheumatic and Immunologic Diseases 2006

ES Molloy, Hoffman GS. Pusey C, J Mason,eds.Large and medium vessel vasculitis


– clinical features and treatment. In The Kidney in Systemic Autoimmune Diseases.
Elsevier Saunders. In press.

Gota C, Mandell BF. Fitzpatrick et al., eds Systemic Vasculitis. In: Textbook of
Dermatology in General Medicine. New York: McGraw Hill. In press.

Mandell BF, Hoffman GS. Rheumatic diseases and the cardiovascular system. In:
Braunwald/Zipes/Libby, eds. Heart Disease: A Textbook of Cardiovascular Medicine. In
press.

Calabrese LH, Kirchner E, Shrestha R. Rheumatic Complications of human


immunodeficiency virus (HIV) infection in the era of highly active antiretroviral therapy
(HAART): emergence of a new syndrome of immune reconstitution and changing
patterns of disease. Sem Arthritis Rheum 2005; 35:166-174.

Calabrese LH, Zein N, Vassilopoulos D. Hepatitis B (HBV) reactivation with


immunosuppressive therapy in rheumatic diseases. Ann Rheum Dis 2006 [Epub ahead
of print].

Chatterjee S, Dombi GW, Severson RK, Mayes MD. Risk of malignancy in scleroderma:
a population-based cohort study. Arthritis Rheum 2005;52:2415-2424.

Hashkes PJ, Laxer RM. Medical treatment of juvenile idiopathic arthritis. JAMA
2005;294:1671-1685.

Uziel Y, Butbul-Aviel Y, Barash J, Padeh S, Mukamel M, Gorodnitski N, Brik R, Hashkes


PJ. Recurrent transient synovitis of the hip in childhood: the long-term outcome among
39 patients. J Rheumatol 2006;33:810-811.
Rheumatic and Immunologic Diseases | 53

Oliveira S, Ravelli A, Pistorio A, Castell E, Malattia C, Prieur AM, Saad-Magalhaes C,


Murray KJ, Sang-Cheol B, Joos R, Foeldvari I, Duarte C, Wulffraat N, Lahdenne P,
Dolezalova P, de Inocencio J, Pratsidou-Gertsi P, Hofer M, Nikishina I, Ozgogan H,
Hashkes P, Martini A, Ruperto N for the Pediatric Rheumatology International Trials
Organization (PRINTO). Health-related quality of life of patients with juvenile idiopathic
arthritis: The PRINTO multinational quality of life cohort study. Arthritis Rheum
2007;57;35-43.

Karlson, EW, Costenbader, KH, McAlindon TE. Massarotti EM, Fitzgerald LM, Jajoo
R, Husni ME, Wright EA., Pankey H, and Fraser PA. High Sensitivity, specificity and
predictive value of the connective tissue disease screening questionnaire (CSQ) among
urban African-American women. Lupus 2005;14:832-836.

Solomon DH, Chibnik LB, Losina E, Huang J, Fossel AH, Husni ME, Katz JN.
Development of a preliminary index that predicts adverse events after total knee
replacement. Arthritis Rheum 2006; May; 54 (5):1536-42.

Stone JH, Hoffman GS, Liota L et al. A serum proteomic approach to gauging the state
of remission in Wegener’s granulomatosis. Arthritis Rheum 2005;52:902-910.

The WGET Research Group. (PI: Stone JH, Co-PI- Hoffman GS). Etanercept plus standard
therapy in patients with Wegener’s granulomatosis. N Engl J Med 2005; 352:351-361.

Merkel PA, Lo GH, Holbrook JT, Tibbs AK, Allen NB, Davis JC, Hoffman GS, McCune
J, St. Clair EW, Specks U, Spiera R, Petri M, Stone JH for The WGET Research Group.
Incidence of venous thrombotic events among patients with Wegener’s granulomatosis.
Ann Intern Med 2005;142:620-626.
54 | Rheumatic and Immunologic Diseases 2006

Seo P, Min YI, Holbrook JT, Hoffman GS, Merkel PA, Spiera R, Davis JC, Ytterberg SR,
St Clair EW, McCune WJ, Specks U, Allen NB, Luqmani RA, Stone JH. Damage caused
by Wegener’s granulomatosis and its treatment: prospective data from the Wegener’s
Granulomatosis Etanercept Trial (WGET). Arthritis Rheum 2005;28;52:2168-2178.

Hoffman GS, Cid MC, Rendt KE, Merkel PA, Weyand CM, Stone JH, Salvarani C, Xu
W, Visvanathan S, Rahman MU for the Infliximab-GCA study group. Prednisone and
infliximab for giant cell arteritis: a randomized, double-blind, placebo-controlled,
multicenter study of efficacy and safety. Ann Intern Med 2007. 146(9):621-630.

Stone JH, Holbrook JT, Marriott MA, Tibbs A, Sejismundo LP, Min Y-I, Specks U, Merkel
PA, Spiera R, Davis JC, St. Clair EW, McCune J, Ytterberg SR, Allen NB, and Hoffman
GS for the WGET Research Group. Solid malignancies in the Wegener’s Granulomatosis
Etanercept Trial. Arthritis Rheum 2006; 54:1608-1618.

Wung PK, Holbrook JT, Hoffman GS, Tibbs AK…. Stone JH for the WGET Research
Group. Herpes Zoster in immunocompromised patients. Incidence, timing and risk
factors. Am J Med 2005; 118: 1409-1416.
Rheumatic and Immunologic Diseases | 55

Lee MS, Smith SD, Galor A, Hoffman GS. Antiplatelet and anticoagulant therapy in
patients with giant cell arteritis. Arthritis Rheum 2006;54:3306-3309.

Deal C, Omizo M, Schwartz,E, et al.Combination teriparatide and raloxifene therapy for


postmenopausal osteoporosis: Results from a 6-month double-blind placebo-controlled
trial. J Bone Miner Res 2005;20:1905-1911.

Harrington T, Deal C. Successes and failures in improving osteoporosis care after


fragility fracture: results of a multi-site clinical improvement project. Arthritis Rheum
(Arthritis Care & Research) 2006;55:724-728.

Deal C, Barr W, Harrington T, Hooker R, Hogan P, Bouchery E, Birnbaum N for the ACR
Workforce Subcommittee COTW7. U.S. Rheumatologist Supply and Demand: 2005-
2006 Workforce Study. Arthritis Rheum 2007;56:722-729.

Calabrese LH, Dodick DW, Schwendt T, Singhal A. Narrative review: reversible cerebral
vasoconstrictive syndromes. Ann Intern Med 2007;146:34-44.
56 | Rheumatic and Immunologic Diseases 2006

Staff Listing | Chairman


Gary S. Hoffman, M.D., M.S.
Chairman, Department of Rheumatic and Immunologic
Diseases

Appointed: 1992

Medical School: Virginia Commonwealth University Medical


College, Richmond, Va.

Specialty Training: Residency – Dartmouth Medical School


and Mary Hitchcock Memorial Hospital, Hanover, N.H.;
Fellowship – Dartmouth Medical School and Mary Hitchcock
Memorial Hospital, Hanover, N.H.; National Institutes of Health,
NIAID, Vasculitis

Other Education: B.A. – State University of New York at


Binghamton, Binghamton, N.Y.; M.S. – Howard University,
Washington, D.C.

Specialty Interests: Vasculitis


Rheumatic and Immunologic Diseases | 57

Staff Listing |
Chairman
Gary S. Hoffman, M.D., M.S.

Quality Review Officer


Chad Deal, M.D.

Center for Osteoporosis and


Metabolic Bone Diseases
Chad Deal, M.D., Director
Abby Abelson, M.D., Education Director
John Carey, M.D., M.S.
Elizabeth File, M.D.
Angelo Licata, M.D., Ph.D., Clinical Trials Director

General Rheumatology
Matthew Bunyard, M.D., Director of Clinical Operations
Abby Abelson, M.D., Education Program Director
John Carey, M.D., M.S.
Soumya Chatterjee, M.D., M.S.
John Clough, M.D.
Carmen Gota, M.D.
Rula Hajj-Ali, M.D.
Elaine Husni, M.D., M.P.H.
Anna Koo, M.D.
Brian Mandell, M.D., Ph.D.
Daniel Mazanec, M.D., Head Center for the Spine
Raymond Scheetz, M.D.
William Wilke, M.D.
58 | Rheumatic and Immunologic Diseases 2006

Section of Clinical Immunology


Leonard Calabrese, D.O., Section Head
Elizabeth Kirchner, C.N.P.

Section of Pediatric Rheumatology


Philip Hashkes, M.D., MSc. Section Head
Steven Spalding, M.D.

Center for Vasculitis Care


and Research
Carol Langford, M.D., M.H.S., Director
Leonard Calabrese, D.O.
Carmen Gota, M.D.
Rula Hajj-Ali, M.D.
Gary Hoffman, M.D., M.S., Chairman
Tiffany Clark, C.N.P.

Center for Clinical Research


Debora Bork, M.F.A., Administrator
Sonya Crook, R.N.
Louise Kincade, R.N.
Katherine Tuthill, C.N.P.
Rheumatic and Immunologic Diseases | 59

Regional Medical Practices


Feyrouz Al-Ashkar, M.D. – Lorain
Chad Deal, M.D. – Solon
Rajul Desai, M.D. M.P.H. – Solon
Howard Epstein, M.D. – Beachwood
Elizabeth File, M.D. – Strongsville
Janice Granieri, M.D., Ph.D. – Willoughby
Stephen MacIntyre, M.D., Ph.D. – Westlake
Judith Manzon, M.D. – Westlake
Susan Mathai, M.D. – Westlake
Alla Modell, M.D. – Independence
Denise Smith Hauser, C.N.P. – Independence
Rochelle Rosian, M.D. – Solon
Jeffrey Wisnieski, M.D. – Willoughby
60 | Rheumatic and Immunologic Diseases 2006

Department Contacts | How to Refer Patients

For Hospital Transfers or Physician Consults

800.553.5056

24 hours a day, seven days a week

Main Campus
9500 Euclid Avenue – Desk A50
Cleveland, Ohio 44105

Appointment Office: 216.444.5632

Financial Counselor: 216.444.3665

Research Office: 216.445.8533

Toll-Free Number: 800.223.2273

www.clevelandclinic.org/arthritis
Rheumatic and Immunologic Diseases | 61

Locations
Willoughby Hills
Lake Erie

Cleveland
Cleveland Clinic
Westlake
Beachwood
Lorain

Independence Solon

Strongsville

Main Campus Solon Family Health Center


9500 Euclid Avenue – Desk A50 29800 Bainbridge Road
Cleveland, Ohio 44105 Solon, Ohio 44139
216.444.5632 440.519.6800
Toll Free Number: 800.648.0022
Rheumatology Regional
Medical Practices
Strongsville Family Health and
Beachwood Family Health and Surgery Center
Surgery Center 16761 Southpark Center
26900 Cedar Road Strongsville, Ohio 44136
Beachwood, Ohio 44122 440.878.2500
216.839.3000
Westlake Family Health Center
Independence Family Health Center 30033 Clemens Road
5001 Rockside Road Westlake, Ohio 44145
Independence, Ohio 44131 440.899.5555
216.986.4000 Toll Free Number: 800.599.7771
Toll Free Number: 800.544.6333
Willoughby Hills Family Health Center
Lorain Family Health Center 2570 SOM Center Road
5700 Cooper Foster Park Road Willoughby Hills, Ohio 44094
Lorain, Ohio 44053 440.943.2500
440.204.7400
Toll Free Number: 800.272.2676
62 | Rheumatic and Immunologic Diseases 2006

Cleveland Clinic Overview |


Cleveland Clinic, founded in 1921, is a not-for-profit academic medical center
that integrates clinical and hospital care with research and education. Today,
1,700 Cleveland Clinic physicians and scientists practice in 120 medical
specialties and subspecialties.

Cleveland Clinic’s main campus, with 41 buildings on 130 acres in Cleveland,


Ohio, includes a 1,000-bed hospital, outpatient clinic, subspecialty centers and
supporting labs and facilities. Cleveland Clinic also operates 13 family health
centers, eight community hospitals, two affiliate hospitals, and a medical facility
in Weston, Florida.

At the Cleveland Clinic Lerner Research Institute, hundreds of principal investigators,


project scientists, research associates and postdoctoral fellows are involved in
laboratory-based research. Total annual research expenditures exceed $150 million
from federal agencies, non-federal societies and associations, and endowment
funds. In an effort to bring research from bench to bedside, Cleveland Clinic
physicians are involved in more than 2,400 clinical studies at any given time.

In September 2004, Cleveland Clinic Lerner College of Medicine of Case Western


Reserve University opened and will graduate its first 32 students as physician-
scientists in 2009.

For more details about Cleveland Clinic, visit clevelandclinic.org


Rheumatic and Immunologic Diseases | 63

Online Services |
eCleveland Clinic

eCleveland Clinic uses state-of-the-art digital information systems to offer several


services, including remote second opinions through a secure Web site to patients
around the world; personalized medical record access for patients; patient
treatment progress access for referring physicians (see below); and imaging
interpretations by the Department of eRadiology’s subspecialty trained academic
radiologists. For more information, please visit eclevelandclinic.org.

DrConnect

Online Access to Your Patient’s Treatment Progress

Whether you are referring from near or far, our new eCleveland Clinic service,
DrConnect, can streamline communication from Cleveland Clinic physicians
to your office. This new online tool offers you secure access to your patient’s
treatment progress at Cleveland Clinic. With one-click convenience, you can
track your patient’s care using the secure DrConnect Web site. To establish a
DrConnect account, visit eclevelandclinic.org or e-mail drconnect@ccf.org.

MyConsult

MyConsult Remote Second Medical Opinion is a secure, online service providing


specialist consultations and remote second medical opinions for more than 600
life-threatening and life-altering diagnoses. MyConsult remote second medical
opinion service allows you to gather information from nationally recognized
specialists without the time and expense of travel. For more information,
visit eclevelandclinic.org/myconsult, e-mail eclevelandclinic@ccf.org or call
800.223.2273, ext 43223.
64 | Rheumatic and Immunologic Diseases 2006

Cleveland Clinic Contact Numbers |


How to Refer Patients
24/7 Hospital Transfers or Physician Consults
800.553.5056

General Information
216.444.2200

Hospital Patient Information


216.444.2000

Patient Appointments
216.444.2273 or 800.223.2273

Medical Concierge
Complimentary assistance for out-of-state patients and families
800.223.2273, ext. 55580, or email: medicalconcierge@ccf.org

International Center
Complimentary assistance for international patients and families
216.444.6404 or visit www.clevelandclinic.org/ic

Cleveland Clinic in Florida


866.293.7866

www.clevelandclinic.org
Cleveland Clinic is determined to exceed the expectations of patients,

families and referring physicians. In light of this goal, we are committed

to providing accurate and timely information about our patient care.

Through participation in national initiatives, we support transparent public

reporting of healthcare quality data and participate in the following public

reporting initiatives:

• Joint Commission Performance Measurement Initiative (www.qualitycheck.org)

• Centers for Medicare and Medicaid (CMS) Hospital Compare


(www.hospitalcompare.hhs.gov)

• Leapfrog Group (www.leapfroggroup.org)

• Ohio Department of Health Service Reporting (www.odh.state.oh.us)

In addition, this publication was produced to assist patients and referring

physicians in making informed decisions. To that end, information about

care and services is provided, with a focus on outcomes of care. For

more information, please visit the Cleveland Clinic Quality Web site at

clevelandclinic.org/quality.
Cover photograph by Stephen Travarca

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