This commissioned review article was subject to full peer-review.
Review article: autoimmune pancreatitis – management of an emerging disease
Article first published online: 8 DEC 2010
© 2010 Blackwell Publishing Ltd
Alimentary Pharmacology & Therapeutics
Volume 33, Issue 3, pages 291–303, February 2011
How to Cite
Kalaitzakis, E. and Webster, G. J. M. (2011), Review article: autoimmune pancreatitis – management of an emerging disease. Alimentary Pharmacology & Therapeutics, 33: 291–303. doi: 10.1111/j.1365-2036.2010.04526.x
- Issue published online: 4 JAN 2011
- Article first published online: 8 DEC 2010
- Publication data Submitted 7 September 2010 First decision 26 September 2010 Resubmitted 13 November 2010 Accepted 14 November 2010EV Pub Online 8 December 2010 EV Pub Online 8 December 2010
Aliment Pharmacol Ther 2011; 33: 291–303
Background Autoimmune pancreatitis is a steroid-responsive inflammatory pancreatic disease considered to be part of an immunoglobulin G4 (IgG4)-associated systemic disease.
Aim To review the management of autoimmune pancreatitis.
Methods We conducted a PubMed search using the following key words: autoimmune pancreatitis, IgG4-associated systemic disease, IgG4-associated cholangitis, diagnosis, natural history, treatment.
Results Although there are reports of spontaneous resolution of autoimmune pancreatitis, steroids have been shown to be effective in inducing remission, reducing the frequency of relapse and that of long-term unfavourable events compared to historical controls. There are no randomised data on autoimmune pancreatitis treatment. Oral steroids are used for induction of remission. Reported response results are excellent with variable proportions of patients achieving remission in different studies. After a period of 2–4 weeks, steroids are tapered and usually withdrawn within several months, although long-term maintenance therapy for all autoimmune pancreatitis patients has also been proposed. Disease relapse occurs in more than 40% of patients and can be effectively treated with additional immunosuppression, including azathioprine.
Conclusions Steroids are effective in inducing remission and in treating relapse in patients with autoimmune pancreatitis. Randomised trials on autoimmune pancreatitis therapy are lacking. To date, questions concerning the timing, choice and duration of long-term immunosuppression remain unanswered.