End-stage renal disease from glomerulonephritis associated with anti-phospholipid syndrome

Byline: Lavjay Butani (1) Keywords: Anti-phospholipid syndrome; Rapidly progressive glomerulonephritis; Chronic renal failure; Thrombotic microangiopathy; Lupus anticoagulant Abstract: Primary anti-phospholipid syndrome (APS) is perceived to be an uncommon disorder, infrequently recognized as a cause of renal disease in childhood. While renal involvement in APS classically manifests as thrombotic events, other renal diseases associated with APS have been reported in adults, including membranous nephropathy and minimal change disease. We report our experience of caring for a child who presented with acute anuric renal failure due to anti-neutrophilic cytoplasmic antibody-negative rapidly progressive glomerulonephritis (RPGN), with concomitant thrombotic microangiopathy (TMA). Recognition of the APS as a cause of the patient's TMA facilitated institution of anticoagulation. Our patient's renal failure did not improve and the patient remained dependent on dialysis until he was successfully transplanted. The purpose of our report is to make health-care professionals aware of the previously unreported association of pauci-immune RPGN and APS in children early recognition of APS will allow initiation of anticoagulation to prevent recurrent thromboses and enable successful transplantation. Author Affiliation: (1) Section of Pediatric Nephrology, University of California Davis Medical Center, 2516 Stockton Boulevard, Ticon II, 3rd Floor, Sacramento, CA 95817, USA Article History: Registration Date: 26/03/2004 Received Date: 29/12/2003 Accepted Date: 10/03/2004 Online Date: 06/05/2004