title: Acute pancreatitis as the first presentation of Wegener's granulomatosis.
abstract: BACKGROUND
  Wegener's granulomatosis is a systemic vasculitis with prominent involvement of the respiratory tract and kidney. An association between acute pancreatitis and Wegener's granulomatosis is rarely reported and is even rarer as the first presentation. This can result in diagnostic difficulty and may allow severe pancreatitis to develop with potentially poor outcome.
  
  
  METHODS
  We report a rare case with fatal outcome of vasculitis consistent with Wegener's granulomatosis presenting as acute pancreatitis in a 20-year-old female. The patient was admitted with worsening abdominal pain associated with nausea and loss of appetite. Accepted causes of acute pancreatitis were excluded and granulomatous vasculitis of the pancreas was confirmed from immunological profile, computed tomography and histology. As the disease progressed the patient experienced cutaneous, pulmonary, renal and severe gut involvement. Thirteen months from diagnosis the patient died of multi-organ failure despite appropriate surgical and immunosuppressive therapy.
  
  
  CONCLUSIONS
  Vasculitic disease of the pancreas is rare but should be considered when other causes have been appropriately ruled out. Careful radiological, immunological and histological diagnosis is necessary and early immunosuppressant therapy in conjunction with advice from immunologists is essential to avoid the poor outcome reported in this and other case reports.
authors.affiliation: Hepatobiliary, Pancreatic and Laparoscopic Surgical Unit, Southampton University Hospital, Southampton, United Kingdom. abu_hlal@yahoo.com
authors.forename: Mohammed
authors.initials: M
authors.lastname: Abu-Hilal
authors.forename: Mohammad
authors.initials: M
authors.lastname: Abu-Hilal
authors.forename: Mark J W
authors.initials: MJ
authors.lastname: McPhail
authors.forename: Bashar
authors.initials: B
authors.lastname: Zeidan
authors.forename: Tim
authors.initials: T
authors.lastname: Bryant
authors.forename: Adrian
authors.initials: A
authors.lastname: Bateman
authors.forename: Colin D
authors.initials: CD
authors.lastname: Johnson
mesh_terms: D000208:Acute Disease; D000328:Adult; D017809:Fatal Outcome; D005260:Female; D014890:Granulomatosis with Polyangiitis / Q000150:complications / Q000175:diagnosis*; D006801:Humans; D010195:Pancreatitis / Q000175:diagnosis* / Q000209:etiology