VALVULAR DYSFUNCTION IN ANTIPHOSPHOLIPID SYNDROME - PREVALENCE, CLINICAL-FEATURES, AND TREATMENT

Valvular abnormalities develop in 36% and 35% of patients with primary antiphospholipid syndrome (PAPS) and with systemic lupus erythematosu s (SLE) respectively, and in 48% of patients with SLE and antiphosphol ipid antibodies (aPL). Valvulopathy includes leaflet thickening, veget ations, regurgitation, and stenosis. A literature survey shows that si gnificant morbidity from valvular dysfunction, mostly mitral regurgita tion leading to congestive heart failure, occurs in 4% and 6% of SLE a nd PAPS patients, respectively. The pathogenesis of valvulopathy may i nvolve interaction of aPL with antigens on the valve surface, resultin g in valvulitis. Current therapy includes symptomatic measures and val ve replacement. A novel approach for symptomatic antiphospholipid synd rome (APS) related valvulopathy involves treatment with systemic corti costeroids. We describe four such patients and their dramatic clinical and hemodynamic response to treatment with prednisone when symptomati c measures failed. Copyright (C) 1997 by W.B. Saunders Company.