SYSTEMIC LUPUS-ERYTHEMATOSUS COMPLICATED BY THROMBOTIC MICROANGIOPATHY

Seven patients with systemic lupus erythematosus (SLE) or SLE-like dis ease developed thrombotic microangiopathy. Prominent features of their acute illnesses were microangiopathic hemolytic anemia (7), thrombocy topenia (7), fever (1), nervous system disease (4), and renal dysfunct ion (5). Laboratory data were significant for antinuclear antibody (AN A) (7), DNA (5), low C3 level (3), low C4 level (2), antiphospholipid antibody (6), schistocytes (7), and lactate dehydrogenase > 500 (7). A ll seven patients received treatment that initially included steroids but later included cyclophosphamide (4), plasma infusion (1), plasmaph eresis (5), intravenous gamma-globulin (2), anti-platelet agents (2), or vincristine (3). Six patients improved, and one patient expired dur ing treatment. Of the six patients who survived this complication, thr ee expired within the year following their acute illnesses. Histology, available in two cases, showed vascular changes consistent with a mic roangiopathic process. We conclude that the spectrum of vascular disea ses in SLE extends beyond vasculitis to include noninflammatory vascul ar processes that can cause equally devastating complications. Copyrig ht (C) 1994 by W.B. Saunders Company