262 SLE AND APS: SOMETIMES A CATASTROPHIC COMBINATION C. Noronha1, A. Galrinho2, A. Panarra1, M. Sousa1, M.J. Barros1, M. Vaz Riscado1, 1 Department of Medicine II, 2Vascular Intervention Unit, Curry Cabral Hospital, Lisbon, Portugal The authors present the case of a 19 year-old Caucasian girl referred in May 1999, due to Systemic Lupus Erythematosus (SLE) based on erythematous skin lesions, fotossensitivity, alopecia, livedo reticularis, polyarthralgias, leucopenia and positive ANA and anti-DNAds, with a three year evolution. Anticardiolipin (IgG) and anti-b2GP1 (IgG), in medium titers, were also detected. No renal involvement was documented. There was a good clinical response to Prednisolone (40 mg), hydroxichloroquine, NSAIDs and acetylsalicilic acid, allowing gradual tapering of prednisolone to 5 mg /d. In November 2000, she presented with left femoro-popliteal phlebothrombosis, configuring a secondary antiphospholipid syndrome (SAPS); improvement was seen following high-intensity oral anticoagulation and corticosteroid increment. The echocardiogram performed then revealed mild mitral insufficiency and verrucous vegetations, interpreted initially as Liebmann- Sacks endocarditis. During the next 4 months, rapidly progressive hemato- proteinuria (to nephrotic range –7,3 g/24h) and edema were noted. Active diffuse prolipherative glomerulonephritis was diagnosed and immunossupression with pulses of Methylprednisolone and IV Cyclophosphamide was begun. Concomitantly, there were signs of heart failure and marked echocardiographic deterioration – severe mitral insufficiency and mild tricuspid insufficiency. This case illustrates the difficulty in management of SLE with SAPS due to multiple organ involvement and the need for multidisciplinary approach. Particularly in this case, despite favorable evolution, several factors were considered (timing of cardiac valve surgery, discontinuation of immunossupression and risks of anticoagulation) and various complications occurred (acute renal insufficiency requiring emergency dyalisis and post-operative cardiac tamponade). Moreover, the authors emphasize the physiopathological role of APS in cardiac valvular lesions.
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