Primary antiphospholipid syndrome presented by total infarction of right kidney with nephrotic syndrome

We report the case of a young woman with primary antiphospholipid syndrome (APS), which presented with acute renal failure, hypoproteinemia, hypoalbum inemia and nephrotic proteinuria. Investigations showed total infarction of right kidney by extensive arterial and vein thrombosis and presence of ant icardiolipin antibodies IgG isotype (anti-beta(2)-glycoprotein I-positive). She was submitted to right nefrectomy and initiated anticoagulant therapy. After nefrectomy, the postoperative period was marked by the development o f arterial hypertension and persistence of nephrotic syndrome. Hypertension was treated with antihypertensive drugs (IECA, beta-blocker and calcium an tagonist). As the nephrotic syndrome persisted despite anticoagulant and an tihypertensive therapy, the patient was treated with oral corticosteroids. Her renal function improved, hypoproteinemia and hypoalbuminemia corrected to normal values and proteinuria decreased to subnephrotic value. We discus s the unusual presentation of this case of primary antiphospholipid syndrom e with total unilateral renal thrombosis and nephrotic syndrome that respon d to anticoagulant, antihypertensive and corticosteroid therapy.