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Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Lecture Objectives:
Identify the spectrum of the vasculitic diseases Recognize the clinical manifestations of vasculitis Develop a diagnostic approach to vasculitis
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Blood vessel damage Thickening of vessel wall Luminal narrowing or occlusion Attenuation of vessel wall
Vessel wall thinning Aneurysm formation or Disruption of the vessel wall with hemorrhage into tissue
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Disruption of the vessel wall with red blood cell extravasation into tissue
Palpable Purpura
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Primary Vasculitides
Unique disease entities without a currently identified underlying cause where vasculitis forms the pathological basis of tissue injury Giant cell arteritis Takayasus arteritis Kawasaki disease Polyarteritis nodosa Wegeners granulomatosis Microscopic polyangiitis ChurgChurg-Strauss syndrome Henoch-Sch Henoch-Schnlein purpura
Secondary Vasculitides
Vasculitis occurring secondary to an underlying disease or exposure
Medications Infection Malignancy Transplant Cryoglobulinemia Connective tissue disease (Rheumatoid arthritis, SLE Inflammatory myositis)
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Diagnosis:
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Aorta
Capillary
Vein
Large Vessel
Giant cell arteritis Takayasus arteritis
Medium Vessel
Polyarteritis nodosa Kawasaki disease
Small Vessel
Wegeners granulomatosis Microscopic polyangiitis Churg-Strauss syndrome Henoch-Schnlein purpura Isolated cutaneous vasculitis
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Aortic aneurysm
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Physical examination:
Nodular, tender, temporal artery with diminished or absent pulsation Scalp ischemia Absent peripheral pulses, asymmetry of blood pressure in extremities
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Diagnosed by:
Temporal artery biopsy
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Aspirin 81 mg daily
- May reduce cranial ischemic complications - Use in all patients without contraindications
Courtesy of Carol A. Langford
Outcome:
70% relapse require increased prednisone 35-85% have prednisone side effects 35 Overall no difference in survival 18x higher rate of thoracic aortic aneurysms - May occur as a late complication of disease - Associated with a high mortality rate
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Clinical Features:
Often presents with acute severe disease
Systemic features Nerve Renal GI tract Digital infarction Fever, weight loss, arthralgias, night sweats Mononeuritis multiplex (ie: foot drop, wrist drop) Hypertension, infarction Pain, infarction, perforation, bleeding Ischemic finger / toe
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Polyarteritis Nodosa
Laboratories:
Elevated ESR Anemia, mild elevations in WBC and platelets May see LFT abnormalities, microscopic hematuria A PAN-like vasculitis can occur with hepatitis B or C PAN-
Diagnosed by:
Arteriography, biopsies
Treatment:
Prednisone + cyclophosphamide Prednisone alone for non-severe disease nonAntiviral treatment if hepatitis B or C are present
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Alveolar hemorrhage
Glomerulonephritis
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Sinus (>95%)
Lung (85%)
Kidney (80%)
20% at diagnosis 80% during course
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Proteinase 3 (PR3)
Myeloperoxidase (MPO)
PR3PR3-ANCA
Wegeners granulomatosis Microscopic polyangiitis ChurgChurg-Strauss syndrome 7575-90% 1010-50% 3-20%
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Wegeners Granulomatosis
Diagnosed by:
Typically biopsy
Treatment:
Outcome:
Survival:
Untreated: 5 months Treatment: 80% survival
Challenges:
Damage from prior disease Treatment related toxicity 5050-70% relapse
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Cutaneous vasculitis represents the most common vasculitic manifestation encountered in clinical practice
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
< 30% no identified cause or disease outside of the skin Treatment of isolated cutaneous vasculitis:
Do not overtreat: recognize that this is not threatening Avoid the use of toxic immunosuppressive agents
Exam
+ + + +/-
Labs
Imaging
+ + + + + +
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Assessment of Vasculitis
History
Do a complete review of systems
Medications: includes over-the-counter, supplements, recreational drugs over-thePhysical Examination Provides clues of organ involvement, features of an underlying disease Laboratories Urinalysis (if (+) blood, check their creatinine immediately) Always Chemistries (to include creatinine and hepatic) Perform CBC with differential ESR, CRP Systemic vasculitis Connective tissue disease Pursue additional lab testing if Infection there are features that point towards: Neoplasm
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Assessment of Vasculitis
Imaging
Chest imaging (CXR, CT) Perform in all patients with pulmonary symptoms No pulmonary symptoms: obtain imaging if there are other features that suggest a systemic small vessel vasculitis Pursue CT, MRI, arteriography of other sites as clinically indicated
Biopsy
Lesions that looks like vasculitis may not be If disease is outside of the skin, these are usually better locations for biopsy to confirm a systemic vasculitis
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.
Harrisons Lecture Notes Copyright The McGraw-Hill Companies. All rights reserved.