title: Autoimmune pancreatitis: clinical and radiological features and objective response to steroid therapy in a UK series.
abstract: OBJECTIVE
  Most cases of autoimmune pancreatitis (AIP) have been reported from Japan. We present data on a UK series, including clinical and radiological features at presentation, and longitudinal response to immunosuppression.
  
  
  METHODS
  Over an 18-month period, all patients diagnosed in our center with AIP were studied. Endoscopic biliary stenting was performed as required, and patients were treated with prednisolone, with response assessed longitudinally. In cases of disease relapse following steroid reduction, azathioprine was instituted.
  
  
  RESULTS
  Eleven patients met diagnostic criteria for AIP. Diffuse pancreatic enlargement was seen in eight patients (73%), and pancreatic duct strictures in all. Seven patients required biliary stents. Extrapancreatic involvement occurred in all, including intrahepatic stricturing and renal disease. Eight weeks after starting steroids, the median serum bilirubin level had fallen from 38 mumol/L to 11 mumol/L (P= 0.001), and ALT from 97 IU/L to 39 IU/L (P= 0.002). Stents were removed in all cases, with no recurrence of jaundice. Improvements in mass lesions and pancreaticobiliary stricturing occurred in all patients. During a median 18-month follow-up, six patients relapsed, four of whom responded to azathioprine. Two patients discontinued steroids and remained well.
  
  
  CONCLUSIONS
  Extrapancreatic disease was an important feature of AIP in this UK series. Initial response to immunosuppressive therapy was excellent, but disease relapse was common. Optimal long-term management remains to be established.
authors: Department of Gastroenterology, University College Hospital, London, United Kingdom.
authors: Nicholas I
authors: NI
authors: Church
authors: Stephen P
authors: SP
authors: Pereira
authors: Maesha G
authors: MG
authors: Deheragoda
authors: Neomal
authors: N
authors: Sandanayake
authors: Zahir
authors: Z
authors: Amin
authors: William R
authors: WR
authors: Lees
authors: Alice
authors: A
authors: Gillams
authors: Manuel
authors: M
authors: Rodriguez-Justo
authors: Marco
authors: M
authors: Novelli
authors: Edward W
authors: EW
authors: Seward
authors: Adrian R W
authors: AR
authors: Hatfield
authors: George J M
authors: GJ
authors: Webster
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D001379:Azathioprine / Q000627:therapeutic use; D002760:Cholangiopancreatography, Endoscopic Retrograde; D003131:Combined Modality Therapy; D003287:Contrast Media; D018450:Disease Progression; D016099:Endoscopy, Gastrointestinal; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008137:Longitudinal Studies; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D011239:Prednisolone / Q000627:therapeutic use; D015607:Stents; D014057:Tomography, X-Ray Computed; D016896:Treatment Outcome; D006113:United Kingdom / Q000453:epidemiology
chemical_list: D003287:Contrast Media; D007166:Immunosuppressive Agents; D011239:Prednisolone; D001379:Azathioprine