Thrombotic thrombocytopenic purpura (TTP) leading to pseudotumour's autoimmune pancreatitis (AIP): A case report

Abstract Introduction Autoimmune pancreatitisis an idiopathic inflammatory disease that produces pancreatic massesand ductal strictures. This benign disease can be associated with extrapancreatic manifestations including cholangitis, sialadenitis, inflammatory bowel disease or retroperitoneal fibrosis, mediastinal adenopathy, interstitial nephritismainly due to immunoglobulin G4 (Ig G4), and occasional association with other auto-immune diseases. Observation We report a 57-year-old woman who developed thrombotic thrombocytopenic purpura(TTP) and pseudo-tumour's seronegative autoimmune pancreatitis(AIP) type 1. The patient was initially treated with pulse corticosteroids and plasmapheresis; afterwards two cures of i.v. Vincristinwith inadequate response and subsequently with four weekly pulses dose of i.v. Rituximab, leading to full remission. Conclusion This case represents the first report of TTP associated to pseudo-tumour's seronegative AIP type 1 successfully treat by Rituximab.