SYSTEMIC LUPUS ERYTHEMATOSUS

Department of Pediatrics University of North Sumatera Medical Faculty H. Adam Malik Hospital

Systemic Lupus Erythematosus

= SLE = Lupus

A rheumatic disease of unknown cause, is characterized by autoantibodies directed against self-antigens and resulting inflammatory damage to target organs : the kidneys blood cells the central nervous system (CNS)
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Etiology :
unknown hallmark autoantibody production against self-antigens

Clinical manifestations :
fever musculoskeletal : arthralgia, arthritis, tendinitis, myositis rash butterfly rash : include nasal bridge thickened epidermis photosensitive

Systemic lupus erythematosus

Presentation 90% tired, arthritis, arthralgia 80% fever 70% hair loss, anemia, swollen lymph nodes 60% weight loss, malar rash 50% pleuritis, pericarditis, nephritis 40% sun light sensitivity SLE : 4 out of 11 ARA criteria (1982 / 1997) 1 Malar rash 2 Discoid lupus 3 Photosensitivity 4 Oral ulcers 5 Arthritis 6 Serositis (pleuritis or pericarditis) 7 Renal disorders (proteinuria or cellular casts) 8 Seizures or psychosis 9 Hemolytic anemia, leukopenia, lymphopenia or thrombocytopenia 10 Anti-DNA antibody, anti-Sm antibody or antiphospholipid antibody positive 11 Positive antinuclear antibody test (positive ANA)

Clinical manifestations (contd)

mucous membrane vasculitic erythema ulcers serositis pleural, pericardial, peritoneal surfaces hepatosplenomegaly GI manifestations pain, diarrhea, melena, hepatitis neurologic manifestations renal disease hypertension, nephrosis, acute renal failure
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Laboratory findings
antinuclear antibodies (ANA) excellent screening anti-double-stranded DNA specific for lupus CH50, C3, C4 decreased in active disease

Treatment
To support clinical well-being corticosteroid (control symptoms and autoantibody product) : Prednison : 1-2 mg/kg/24hr divided doses, and when complement levels rise to within the normal range, the dose is carefully tapered over 2-3 yr to the lowest effective dose Severe disease cytotoxic therapy : - cyclophosphamide - azathioprine
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