Autoimmune pancreatitis: clinical and radiological features and objective response to steroid therapy in a UK series.
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.
Church
Nicholas I
NI
Department of Gastroenterology, University College Hospital, London, United Kingdom.
Pereira
Stephen P
SP
Deheragoda
Maesha G
MG
Sandanayake
Neomal
N
Amin
Zahir
Z
Lees
William R
WR
Gillams
Alice
A
Rodriguez-Justo
Manuel
M
Novelli
Marco
M
Seward
Edward W
EW
Hatfield
Adrian R W
AR
Webster
George J M
GJ
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
D003287:Contrast Media; D007166:Immunosuppressive Agents; D011239:Prednisolone; D001379:Azathioprine