SYSTEMIC LUPUS SYSTEMIC LUPUS ERYTHEMATOSUSERYTHEMATOSUS

Dr Nadim Iqbal SheikhDr Nadim Iqbal Sheikh

Assoc. Prof. MedicineAssoc. Prof. Medicine

Rawalpindi General HospitalRawalpindi General Hospital

SLESLE

DEFINITION: An inflammatory disease DEFINITION: An inflammatory disease which results in multisystem involvement which results in multisystem involvement and has a varied clinical presentationand has a varied clinical presentation

– No uncommonNo uncommon

– Male:female ratio 1:10Male:female ratio 1:10

– Most common in African American women Most common in African American women (1:250)(1:250)

Predisposing factorsPredisposing factorsHLA DR3HLA DR3Complement deficiencyComplement deficiencyIncreased Oestrogens or reduced androgensIncreased Oestrogens or reduced androgensDrugs, virusesDrugs, viruses

Tissue Damage

AutoantibodyProduction

Immune complexesIncreased

B cell stimulation

Reduced TCell function

Drugs Causing SLEDrugs Causing SLE

– HydralazineHydralazine– ProcainamaideProcainamaide– QuinidineQuinidine– PhenytoinPhenytoin– IsoniazidIsoniazid– chlorpromazinechlorpromazine

Drug Induced SLEDrug Induced SLE

– HydralazineHydralazine 50% may develop ANA, only 10% 50% may develop ANA, only 10%

develop lupus like diseasedevelop lupus like disease

– Anti ds DNA usually absentAnti ds DNA usually absent– Cerebral and renal invovement rareCerebral and renal invovement rare– Antihistone antibodies in 95%Antihistone antibodies in 95%– Complement deficiencies are Complement deficiencies are

uncommonuncommon

Non-organ-specific antibodies Non-organ-specific antibodies and their frequency in SLEand their frequency in SLE

Anti ds DNAAnti ds DNA Highly specific for SLEHighly specific for SLE 90%90%

Ant ss DNAAnt ss DNA Non specificNon specific 60%60%

Anti-nRNPAnti-nRNP Low titre in SLE, high titre in MCTDLow titre in SLE, high titre in MCTD 40%40%

Anti Sm Anti Sm More common in blacksMore common in blacks25%25%

Anti La(SSB)Anti La(SSB) Sjogrens syndromeSjogrens syndrome 15%15%

Anti SLAnti SL Fever and lymphadenopathyFever and lymphadenopathy 8%8%

Anti DNA histoneAnti DNA histone 95% in drug induced SLE95% in drug induced SLE 50%50%

Anticytoplasmic antibodiesAnticytoplasmic antibodies

Anti Ro(SSA)Anti Ro(SSA) ANA negative SLE andjogren’s SyndromeANA negative SLE andjogren’s Syndrome

40%40%

ARA Criteria for Dx of ARA Criteria for Dx of SLESLE CriteriaCriteria %%

– Malar rashMalar rash 6262– Discoid RashDiscoid Rash 3030– PhotosensitivityPhotosensitivity 1616– Oral/nasal ulcersOral/nasal ulcers 2222– Non-deforming arthritisNon-deforming arthritis 8686

CriteriaCriteria %%– Renal DiseaseRenal Disease 2424– Neurological diseaseNeurological disease– (psychosis/seizures)(psychosis/seizures) 1919– Haem(Haem(↓Hb,WBC,Platelets↓Hb,WBC,Platelets 5050

– SerositisSerositis (pleurisy/percarditis)(pleurisy/percarditis) 3030

ARA Revised Criteria 1982ARA Revised Criteria 1982

Drug Therapy for SLEDrug Therapy for SLE

DrugDrug IndicationsIndications– NSAIDsNSAIDs synovitis & mild synovitis & mild

systemic illnesssystemic illness– AntimalarialsAntimalarials synovitis & cutaneous synovitis & cutaneous

diseasedisease– CorticosteroidsCorticosteroids Moderate to severe systemic Moderate to severe systemic

disease disease including vasculitis, including vasculitis, neurpathy, neurpathy, nephritis, nephritis, vasculitis and otyher vital vasculitis and otyher vital organsorgans

– ImmunosuppressantsImmunosuppressants Severe disease including Severe disease including nephritisnephritis

– Plasma ExchangePlasma Exchange Severe vasculitis & nephritisSevere vasculitis & nephritis

Pregnancy and SLEPregnancy and SLE

Fertility is usually normal except in severe Fertility is usually normal except in severe diseasedisease– No major contraindication to pregnancyNo major contraindication to pregnancy– For Contraception Barrier methods rather than pill For Contraception Barrier methods rather than pill

are preferable as Oestrogens can precipitate are preferable as Oestrogens can precipitate relapserelapse

– Recurrent miscarriages occur (?antiphospholipid Recurrent miscarriages occur (?antiphospholipid syndrome)syndrome)

– Postpartum exacerbations are not infrequentPostpartum exacerbations are not infrequent– Continue usual treatmentContinue usual treatment– Control HTN wellControl HTN well– Eetal loss in severe disease and APL syndrome Eetal loss in severe disease and APL syndrome

~25%~25%

PROGNOSISPROGNOSIS

In 1950’s 5 year survival was 50%In 1950’s 5 year survival was 50% In 1990’s 10 year survival is in In 1990’s 10 year survival is in

excess of 90%excess of 90% Patients with renal and Patients with renal and

neurological involvement have neurological involvement have poorer prognosispoorer prognosis

Issues in managementIssues in management

Reduction of steroids any further leads to Reduction of steroids any further leads to joint pains, elevation of ESR and CRP with joint pains, elevation of ESR and CRP with depression of Complement levelsdepression of Complement levels

Safety of hydoxychloroquine with a solitary Safety of hydoxychloroquine with a solitary eyeeye

Counselling regarding marriage and having Counselling regarding marriage and having family (steroids and immunosupressant)family (steroids and immunosupressant)

Anticonvulsants (how long to continue as fits Anticonvulsants (how long to continue as fits are likely to recur in secondary epilepsy)are likely to recur in secondary epilepsy)

UPDATEUPDATE

Fully active, going to collegeFully active, going to college Steroids withdrawnSteroids withdrawn Dose of azathioprine increasedDose of azathioprine increased Eye surgery with placement of Eye surgery with placement of

artificial eye done.artificial eye done.

THANK YOUTHANK YOU