LUPUS

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.