Pediatr Nephrol (2004) 19:812–814 DOI 10.1007/s00467-004-1491-3 BRIEF REPORT Lavjay Butani End-stage renal disease from glomerulonephritis associated with anti-phospholipid syndrome Received: 29 December 2003 / Revised: 9 March 2004 / Accepted: 10 March 2004 / Published online: 6 May 2004  IPNA 2004 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 pa- tient’s renal failure did not improve and the patient re- mained 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 antico- agulation to prevent recurrent thromboses and enable successful transplantation. Keywords Anti-phospholipid syndrome · Rapidly progressive glomerulonephritis · Chronic renal failure · Thrombotic microangiopathy · Lupus anticoagulant Introduction Primary anti-phospholipid syndrome (APS) is increas- ingly recognized as a cause of spontaneous thrombotic events in adults and in children [1, 2]. A variety of renal manifestations have been reported in patients who have anti-phospholipid (APL) antibodies. Two commonly re- ported renal manifestations of APS are the so-called APS nephropathy and renal arterial/venous thromboses. The former is characterized by the triad of intimal hyperplasia, focal cortical atrophy, and thrombotic microangiopathy (TMA), and results from vaso-occlusive disease in the small intra-renal vasculature [3]. Recently, other ‘non- conventional’ renal manifestations have been recognized in adults with APL antibodies; these include membranous nephropathy, minimal change disease, and rarely pauci- immune anti-neutrophilic cytoplasmic antibody (ANCA)- negative nephritis [4]. However, whether the APL anti- bodies in these settings are epiphenomena or truly con- tributory to the pathogenesis of the renal disease is far from clear. Typically, primary APS is not commonly thought of in children with unexplained acute renal fail- ure, nor is it considered to be a cause of end-stage renal disease (ESRD), either in adults or in children. We report our experience in managing a child who presented with severe anuric renal failure from pauci- immune necrotizing ANCA-negative nephritis with con- comitant intra-renal TMA, which rapidly progressed to ESRD. To our knowledge, this is the first description of a pediatric patient with primary APS and concomitant pauci-immune nephritis, and the first report describing the development of ESRD as a consequence of primary APS. Our report emphasizes the need for health-care profes- sionals to consider primary APS as a possible cause of rapidly progressive glomerulonephritis (RPGN), so that appropriate therapy may be instituted early. Case report A 4-year-old Caucasian boy was transferred from a community hospital to the University of California Davis Medical Center Pe- diatric Intensive Care Unit for management of renal failure. He had presented to the local emergency department (ED) with a 1-week history of low-grade fever and rhinorrhea, both of which had re- solved. Five days prior to admission he had complained of severe headache and was noted to be listless. This progressed to the de- velopment of vomiting, high fever, and a cough on the day of admission. In the ED the patient was hypertensive (blood pressure L. Butani ( ) ) Section of Pediatric Nephrology, University of California Davis Medical Center, 2516 Stockton Boulevard, Ticon II, 3rd Floor, Sacramento, CA 95817, USA e-mail: lavjay.butani@ucdmc.ucdavis.edu Tel.: +1-916-7348118 Fax: +1-916-7340629