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TammyRascicFNPS
WhatisSystemicLupusErythematosus
SystemicLupusErythematosus(lupus)isarare
autoimmunediseaseofanunknownetiology
Characterizedbyachronicrelapsingcoursethatcan
bemildtosevere
Itusuallyaffectstheskin,jointsandmucous
membranes
Lesscommonlyitcaneffectmultiorgansystem
complicationssuchasrenal,hematologicaland
neurological
Epidemiology&Incidence
Predominantageis1545yearsold
3050/100,000seenyearly
Lupususuallyisseenmoreofteninwomenthanmen
8:1
MostcommonlyeffectsAfricanAmericanwomen8.1
11.4/100,000peryear
LowersocioeconomicstatusandAsianandHispanic
ethnicityislinkedtocomplications,suchasrenal
disease,andhighermortalityrates
RiskFactors
Race:AfricanAmericans,Hispanics,
Asians,andNativeAmericans
Females>Males
Environmentalfactors:UVlight,
infectiousagents,stress,diet,drugs,
hormones,andcigarettesmoke
Etiology
Mostcasesareidiopathic
Geneticandenvironmentalfactors
Druginducedlupus:Hydralazine,
quinidine,procainamide,minocycline,
isoniazid,TNFalphainhibitors,etc
Pathophysiology
Skin:photosensitivity;scalyerythematousplaqueswithfollicular
plugging,dermalatrophyandscarring;nonscarring
erythematouspsoriasiformorannualrash;alopecia;mucosal
layers
Musculoskeletal:Nonerosivearthritis;ligamentandtendon
laxity,ulnardeviation,andswanneckdeformities;avascular
necrosis
Renal:Glomerulonephritis
Pulmonary:Pleuritis,pleuraleffusion,alveolarhemorrhage,
pneumonitis,interstitialfibrosis,shrinkinglung,pulmonary
hypertension,pulmonaryembolism
Pathophysiology(Continued)
Cardiac:Nonbacterialverrucousendocarditis,pericarditis,
myocarditis,atherosclerosis
CNS:Thrombosisofsmallintracranialvesselswithperivascular
inflammationresultinginmicro/macroinfarctswithhemorrhage
Peripheralnervoussystem:Mononeuritismultiplex,peripheral
neuropathy
Gastrointestinal:Pancreatitis,peritonitis,colitis
Hematologic:Hemolyticanemia,thrombocytopenia,leukopenia,
lymphopenia
Vascular:Vasculitis,thromboembolism
EarlyDiagnosisandSymptoms
Earlydiagnosisiskeytopreventseriouscomplications.
Earlysymptomsinclude:
Severefatigue,malaise
Flittingarthraglgias,weaknessandmyalgias
Mouthulcers(usuallypainless)
headaches
Rashes,particularlyprovokedbythesun
Persistentproteinuria
Worseningsystemsduringandpriortomenstration
History
Previousslidesymptomsalongwith:
Weightloss,headache
Pleuriticchestpain,cough,dyspnea,hemoptysis
Stroke,seizure,psychosis,cognitivedefects
Cellularcasts
Hemolyticanemia,anorexia,nausea,vomiting
RaynaudPhenomenon
ClinicalFindings
VitalSigns:fever,hypertension
Malar,discoid,psoriasiform,orannularrash,alopecia
Oralornasalulcers
Lymphadenopathy,splenomegaly
Acrocyanosis
Inflammatoryarthritis,tenosynovitis
Pleuralorpericardialrub,heartmurmur
Bibasilarrales
Cranialorperipheralneuropathies
Diagnostics
InitialLabTests:
Antinuclearantibody(highsensitivity98%,low
specificity)
AntidoublestrandedDNAandantiSmithantibodies
(highspecificityforSLE)
RNAproteinantibodies(lessspecificforSLE)
FalsepositiveVDRL(highsensitivity,lowspecificity)
Lowserumcomplementlevels:C3,C4,Ch50
ESR(nonspecific,oftenhighinactivedisease)
Diagnostics(Continued)
CBC(forhemolyticanemia,thrombocytopenia,
leukopenia,lymphopenia
SerumCreatinine(elevatedinlupusnephritis)
Urinalysis(proteinuria,hematuria,cellularcast)
Phospholipidantibodies
AntiP(ribosomalautoantibodies)are
associatedwithSLEarthritisanddiseaseactivity
Imaging
Initialimagingdependsonpresentingsymptoms
Radiographofinvolvedjoints
Chestxray:infiltrates,pleuraleffusion,lowlungvolumes
ChestCTscan,VQscan,duplexultrasoundforPEorDVT
HeadCT:Ischemia,infart,hemorrhage
BrainMRI:Focalareasofincreasedsignalintensity
Echocardiogram:pericardialeffusion,valvularvegetations,
pulmonaryhypertension
Contrastangiographyformediumsizearteryvasculitis:
Mesentericorlimbischemia,CNSsymptom
Procedures/Surgery
Renalbiopsytodiagnoselupusnephritis
Skinbiopsywithimmunoflurescenceoninvolvedanduninvolved
nonsunexposedareas(mayhelpdifferentiateSLErashfrom
others)
LumbarpunctureinpatientswithfeverandCNSormeningeal
symptoms
EEGforseizuresorglobalCNSdysfunction
Neuropsychiatrictestingforcognitiveimpairment
EMG/NCSforperipheralneuropathyandmyositis
Nerveand/ormusclebiopsy
ECG,cardiacenzymes,stresstests
DifferentialDiagnosis
Undifferentiatedconnectivetissuedisease
Sjogrensyndrome
fibromyalgia
RA
Vasculitis
Idiopathicthrombocytopeniapurpura
Antiphospholipidantibodysyndrome
Druginducedlupus
Social/EnvironmentalConsiderations
Counselingandsupportmaybebeneficial
AvoidUVlightexposure(wearSPF>30
sunscreen&protectiveclothing/hat)
Weightcontrol,smokingcessation,exercise
Stressavoidance/management
PharmacologicManagement
Antimalarialagents(hydroxychloroquine)andNSAIDs
(Motrin)arefirstlinetreatmentsforpatientswithmildSLE
Topicalorintralesionalglucocorticosteriodsforskin
manifestations
Systemicglucocorticoids(prednisoneorequivalent)
Lowdose(<0.5mg/kg)forminordiseaseactivitythatis
notrespondingwelltoNSAIDs
Highdose(12mg/kg/d)orIVmethlyprednisolonefor
organthreateningdisease(likeCNSandrenal)
NonpharmacologicManagement
Biofeedback(processofusinghightech
monitoringequipmenttoprovideinformation
aboutonesbiologicalconditionsuchaslevels)
Visualimagery(mindbodytherapy,formof
hypnosis)
Cognitivetherapy(typeofpsychotherapythat
examinestherelationshipsbetweenthoughts,
feelings,andbehaviors)
Complications
Infections
Neoplasms
CardiacDisease
Nephritis
Neuropsychiatriclupus
Followup
Clinicalevaluationforsignsandsymptoms:
Weeklytomonthlyforactivedisease
Every36monthsformildorinactivedisease
Labstudies:CBCwithdifferential,serumcreatinine,
urinalysis,C3,C4,DSDNA,ESR
Monitorforadverseeffectsoftreatment
Measuresofdiseaseactivityanddamage
Education
Influenza/pneumoniavaccinesaresafe(avoid
livevaccinesinimmunocompromisedpatients)
Lowestrogenoralcontraceptivesaresafein
mildSLE
Nospecialdietunlessforcomplications(renal
failure,diabetes,hyperlipidemia.Needadequate
calcium/vitaminDinpatientstaking
corticosteroids)
Referral
Multiplepossiblereferralsforvariousbodysystemsdependingontheseverity
andhowitisbeingcontrolledintheoffice:
Nephrologist
Neurologist
Dermatology
Immunology
Cardiology
Psychology
Hematology
Rheumatology
Nutritionist
Quiz:Question1
1.)Lupususuallyeffects:
A.)Skin
B.)Joints
C.)MucousMembranes
D.)Alloftheabove
Answer:DAlloftheabove
Quiz:Question2
2.)Lupuslesscommonlyeffectwhatsystem?
A.)Skin,jointsandmucousmembranes
B.)Renal,hematologicalandneurological
C.)Cardiacandintegumentary
D.)Alloftheabove
Answer:BRenal,hematologicalandneurological
Quiz:Question3
3.)Whatisthepredominantageforpeopleusually
diagnosedwithlupus?
A.)1035
B.)1545
C.)2050
D.)3055
Answer:B1545yearsold
Quiz:Question4
4.)Lupusisseenmorecommonlyinwhatsex?
A.)Male
B.)Female
C.)Equalinbothmalesandfemales
D.)None
Answer:BFemale
Quiz:Question5
5.)Whatethnicityislupususuallyseenin?
A.)European
B.)Asian
C.)AfricanAmerican
D.)Hispanic
AnswerCAfricanAmerican
Quiz:Question6
6.)EarlySymptomsoflupusincludeallEXCEPT:
A.)Rash
B.)Mouthulcers
C.)Headaches
D.)hemoptysis
Answer:DHemoptysis
Quiz:Question7
7.)AllareclinicalfindingsoflupusEXCEPT:
A.)Lymphadenopathy
B.)Hypotension
C.)Acrocyanosis
D.)Splenomegaly
Answer:BHypotension
Quiz:Question8
8.)Whichlabresultsarehighisactivelupusdisease?
A.)CD3
B.)CD4
C.)ESR
D.)Ch50
Answer:CESR
Quiz:Question9
9.)WhichisNOTadifferentialdiagnosisforlupus?
A.)Vasculitis
B.)Fibromyalgia
C.)Rheumatoidarthritis
D.)Osteoarthritis
Answer:DOsteoarthritis
Quiz:Question10
10.)Whichtypeofglucocorticosteriodsdoyougivefor
alupuspatientthatisnotrespondingwelltoNSAID
treatmentformildsympotoms?
A.)Topicalglucocorticosteriods
B.)Lowdoseglucocorticosteriods
C.)Highdoseglucocorticosteriods
D.)Intralesionalglucocorticosteriods
Answer:BLowdoseglucocorticosterioids
References
Brown,S.,Bond,D.,&Waldron,N.
(2014).Lesscommonrheumatological
diseases:anintroduction.Practice
Nurse,44(9),4046.
Domino,F.J(2013).The5Minute
ClinicalConsult2013.Massachusetts:
WoltersKluwer,LippincottWilliams&
Wilkins.