Systemic Lupus Systemic Lupus ErythematosusErythematosus

Dr CheeDr Chee--Seng YeeSeng YeeUniversity of BirminghamUniversity of Birmingham

EpidemiologyEpidemiology

�� Female: MaleFemale: Male 99--10:110:1

�� Racial predispositionRacial predispositionfemale: overallfemale: overall 1/20001/2000

AfroAfro--CaribbeanCaribbean 1/5001/500AsianAsian 1/10001/1000CaucasianCaucasian 1/25001/2500

TriggersTriggers

UltraUltra--violet lightviolet light

InfectionInfection

OestrogenOestrogen

StressStress

ImmunologyImmunology

�� Apoptosis problemApoptosis problemdefective clearance defective clearance

�� B cell defectB cell defect

�� T cell defectT cell defect

Clinical FeaturesClinical Features

�� varied manifestationsvaried manifestations

�� any organ systems can be affectedany organ systems can be affected

����������������������������������

ConstitutionalConstitutional

�� FatigueFatigue

�� FeverFever

�� Loss of appetiteLoss of appetite

�� Loss of weightLoss of weight

MusculoskeletalMusculoskeletal�� Arthralgia/MyalgiaArthralgia/Myalgia�� MyositisMyositis�� NonNon--erosive synovitiserosive synovitis�� JaccoudJaccoud’’ss arthropathyarthropathy (reversible (reversible

deformity)deformity)

RenalRenal

�� GlomerunephritisGlomerunephritisProliferativeProliferative: : MesangialMesangial

FocalFocalDiffuseDiffuse

MembranousMembranous

�� Interstitial nephritisInterstitial nephritis

NeuropsychiatricNeuropsychiatric

�� PsychosisPsychosis�� SeizureSeizure�� ConfusionalConfusional statestate�� Cognitive dysfunctionCognitive dysfunction�� Stroke syndromeStroke syndrome�� MyelopathyMyelopathy�� NeuropathyNeuropathy�� Intracranial hypertensionIntracranial hypertension

CardiovascularCardiovascular

�� PericarditisPericarditis�� MyocarditisMyocarditis�� EndocarditisEndocarditis�� VasculitisVasculitis�� RaynaudRaynaud’’ss�� Premature atherosclerosisPremature atherosclerosis

RespiratoryRespiratory

�� PleurisyPleurisy�� FibrosingFibrosing alveolitisalveolitis�� BronchiolitisBronchiolitis obliteransobliterans�� Shrinking lungShrinking lung�� Pulmonary haemorrhagePulmonary haemorrhage�� Pulmonary hypertensionPulmonary hypertension

GITGIT

�� SerositisSerositis�� Lupus enteritisLupus enteritis�� Intestinal Intestinal pseudoobstructionpseudoobstruction�� MalabsorptionMalabsorption�� Protein losing Protein losing enteropathyenteropathy�� HepatitisHepatitis�� CholecystitisCholecystitis�� PancreatitisPancreatitis

EyeEye

�� KeratitisKeratitis�� UveitisUveitis�� ScleritisScleritis�� EpiscleritisEpiscleritis�� Retinal Retinal vasovaso--occlusive diseaseocclusive disease�� Anterior Anterior ischaemicischaemic optic neuropathyoptic neuropathy�� Optic neuritisOptic neuritis

HaematologyHaematology

�� AnaemiaAnaemiaHaemolytic & NonHaemolytic & Non--haemolytichaemolytic

�� LeucopeniaLeucopeniaLymphopeniaLymphopeniaNeutropeniaNeutropenia

�� ThrombocytopeniaThrombocytopenia

AntiphospholipidAntiphospholipid SyndromeSyndrome

�� Venous/Arterial thrombosisVenous/Arterial thrombosis�� Pregnancy morbidityPregnancy morbidity

fetalfetal losslossprepre--eclampsiaeclampsiaintraintra--uterine growth restrictionuterine growth restriction

�� AntiphospholipidAntiphospholipid antibodiesantibodies

LaboratoryLaboratory

�� CRP CRP vsvs ESRESR↑↑ CRP: CRP: synovitissynovitis

serositisserositisinfectioninfection

�� ComplementsComplementsC3/C4C3/C4C3dC3d

AutoantibodiesAutoantibodies

�� AntiAnti--dsDNAdsDNA60 60 –– 70%70%kidney diseasekidney diseasehigh affinity more specifichigh affinity more specific

�� AntiAnti--Ro/LaRo/La20 20 –– 50%50%subacutesubacute & chronic & chronic cutaneouscutaneousneonatal lupus syndromeneonatal lupus syndrome

AutoantibodiesAutoantibodies

�� AntiAnti--SmSm10 10 –– 30%30%? kidney and CNS disease? kidney and CNS disease

�� AntiAnti--histonehistonedrugdrug--induced lupusinduced lupus

TreatmentTreatment

�� EducationEducation�� FertilityFertility

pregnancy planningpregnancy planning

�� SunblockSunblock�� SymptomaticSymptomatic

NSAIDsNSAIDs

TreatmentTreatment

�� AntimalarialsAntimalarials (HCQ, (HCQ, MepacrineMepacrine))�� Corticosteroids (oral/iv/Corticosteroids (oral/iv/im/intralesionalim/intralesional))�� ImmunosuppressivesImmunosuppressives

azathioprineazathioprineciclosporinciclosporin AAmethotrexatemethotrexateMMFMMFcyclophosphamidecyclophosphamide

TreatmentTreatment

�� BiologicalsBiologicalsrituximabrituximab

�� OthersOthersprasteroneprasteronethalidomidethalidomideacitretinacitretin

SurvivalSurvival

5 years5 years 93%93%10 years10 years 85%85%15 years15 years 79%79%20 years20 years 68%68%

AbuAbu--ShakraShakra J Rheum 1995J Rheum 1995

0%10%20%30%40%50%60%70%80%90%

100%

% Survival of SLE patients