SYSTEMIC LUPUS ERYTHEMATOSUS

(SLE)

dr. Made T. Ardhana, SpPD

DEFINITION Disease of unknown etiology in which

tissues and cells are damaged by deposition of pathogenic autoantibodies and immune complexes

Pathogenic importance :Genetic, environmental, and sex hormonal factors

Characteristics :a) T and B cell hyperactivityb) Production of autoantibodies with specificity

for nuclear antigenic determinantsc) Abnormalities of T cell function

CLINICAL MANIFESTATIONS 90% of pts are women, child – bearing

age, more common in blacks than whites Course in disease is often on one of

periods of exacerbation and relative passive

Involve virtually any organ system Wide range of disease severity

COMMON FEATURES Constitutional → fatigue, fever, malaise,

weight loss Cutaneous → rashes, photosensitivity,

vasculitis, alopecia, oral ulcers Arthritis → inflammatory, symmetric,

nonerosive Hematologic → anemia, neutropenia,

lymphadenopathy, thrombocytopenia, splenomegaly, venous or arterial thrombosis

Cardiopulmonary → pleuritis, pericarditis, myocarditis, endocarditis

Nephritis GI → peritonitis, vasculitis Neurologic → organic brain syndromes,

seizures, psychosis, cerebritis

DRUG – INDUCED LUPUS A clinical and immunologic picture similar

to spontaneous SLE May be induced by drugs : procainamide,

hydralazine, isoniazid, chlorpromazine, methyldopa

Features are predominantly constitutional, joint, and pleuropericardial; CNS and renal disease are rare

All pts have antinuclear antibodies (ANA), antihistone antibodies may be present but antibodies to dsDNA and hypocomplementemia are uncommon

Most pts improve following withdrawal of offending drug

EVALUATION Hx and physical exam Presence of ANA is a cardinal factor, but a

(+) ANA is not specific for SLE Laboratory assessment should include :

CBC, ESR, ANA and subtypes (antibodies to dsDNA, ssDNA, Sm, Ro, La, Histone), complement levels (C3, C4, CH50), serum immunoglobulins, VDRL, PT, PTT, anticardiolipin antibody, lupus anticoagulant, UA

Appropriate radiographic studies ECG Consideration of renal biopsy if evidence of

glomerulonephritis

TREATMENT Choice of therapy is based on type

and severity disease manifestations Goals are to control acute, severe

flares, develop maintenance strategies where symptoms are suppressed to acceptable level

Treatment choices depend on :a) Whether disease is life – threatening or

likely to cause organ damageb) Whether manifestations are reversiblec) The best approach to prevent

complications of disease and treatment

Conservative Therapies for Non – Life – Threatening Disease NSAIDs (e.g. ibuprofen 400 – 800 mg tid – qid) Antimalarials (hydroxychloroquine 400 mg/d) –

may improve constitutional, cutaneous, articular manifestations. Ophthalmologic evaluation required before and during Rx to rule out ocular toxicity

Treatments for Life – Threatening SLE Systemic glucocorticoids Anticoagulation - may be indicated in pts with

thrombotic complications Cytotoxic agents – beneficial in active

glomerulonephritis; may be required for severe disease not successfully controlled by acceptable doses of steroids

1) Cyclophosphamide – considered the standard drug for controlling life – threatening active lupus nephritis

2) Mycophenolate motefil – short term studies suggest efficacy in some pts with SLE

3) Azathioprine – indicated in pts who cannot take cyclophosphamide