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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
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