Review article: autoimmune pancreatitis - management of an emerging disease.

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 After a period of 2-4 weeks, steroids are tapered and usually withdrawn within several months, although long-term maintenance therapy for all autoimmune pancreatitis patients has also been proposed.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.