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

Introduction: Autoimmune pancreatitis is an idiopathic inflammatory disease that produces pancreatic masses and ductal strictures. This benign disease can be associated with extrapancreatic manifestations including cholangitis, sialadenitis, inflammatory bowel disease or retroperitoneal fibrosis, mediastinal adenopathy, interstitial nephritis mainly 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. Vincristin with 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.