1366 Primary Antiphospholipid Syndrome, Acute Necrotizing Pancreatitis, and IgA Nephropathy in a Young Male: Single Pathogenesis or Rare Association?

Abstract

INTRODUCTION: Primary Antiphospholipid syndrome (APS) is characterized by recurrent thrombosis, fetal loss and the presence of lupus anticoagulant (LA)/anticardiolipin antibodies. Primary APS is uncommon in males and poses diagnostic difficulties. Primary APS is considered as a rare cause of acute pancreatitis and IgA nephropathy (IgAN). We describe a patient of primary APS (probably the first reported case), who sequentially presented with cerebral venous thrombosis, later with recurrent episodes of acute necrotizing pancreatitis (ANP) followed by IgAN. Despite the initial diagnostic dilemmas, the patient was managed successfully. CASE DESCRIPTION/METHODS: A 30 year old male initially presented with sudden onset headache and generalized seizures. CECT brain revealed cerebral venous thrombosis. Cardiac and Pro thrombotic work up (including ANA and LA) was normal. He was treated with antiepileptics and warfarin. 3 months later, he presented with 3 episodes of acute pancreatitis. Each time the patient was managed conservatively with fluids and analgesics. However the last episode was very severe, CECT abdomen was suggestive of severe ANP with extensive splenic vein thrombosis (SVT). Anti-β2glycoprotein1 antibody test was strongly positive. APS was diagnosed according to revised Sapporo criteria. Low molecular heparin and steroids were started. After 5 months, although the patient recovered from ANP, he presented with hypoproteinemia and renal failure. Urine analysis showed RBC casts and proteinuria. Kidney biopsy showed mesangial IgAN with secondary segmental sclerosis. Patient recovered fully and is on follow up for two years. He is doing fine with low dose steroids, ACE inhibitors and warfarin with regular INR (target 2-3). DISCUSSION: This patient had multiple hospitalizations, all attributable to the rare manifestations of APS. It is perhaps the first case report where APS, ANP and IgAN are described in a single patient. Clinical suspicion can lead to early diagnosis, which makes it easy to manage such patients. Acute pancreatitis is considered as one of the rarest manifestation of APS, which is distinct from autoimmune pancreatitis. Renal biopsy was suggestive of IgAN, which is unusual and distinct from the APS nephropathy. Thrombosis of the pancreatic and renal microcirculation and vasculitis has been postulated as the underlying pathogenesis. To conclude, APS is a rare, yet treatable cause of acute pancreatitis. Urine examination is a must in cases of primary APS, with or without pancreas involvement.