Presentation and Management of Post-treatment Relapse in Autoimmune Pancreatitis/Immunoglobulin G4-Associated Cholangitis

• Published in: Clinical gastroenterology and hepatology Vol. 7; no. 10; pp. 1089 - 1096
• Main Authors: Sandanayake, Neomal S, Church, Nicholas I, Chapman, Michael H, Johnson, Gavin J, Dhar, Dipok K, Amin, Zahir, Deheragoda, Maesha G, Novelli, Marco, Winstanley, Alison, Rodriguez–Justo, Manuel, Hatfield, Adrian R.W, Pereira, Stephen P, Webster, George J.M
• Format: Journal Article
• Language: English
• Published: United States Elsevier Inc 01-10-2009
• Subjects: Adult; Aged; Aged, 80 and over; Anti-Inflammatory Agents - therapeutic use; Autoimmune Diseases - drug therapy; Autoimmune Diseases - pathology; Autoimmune Diseases - physiopathology; Azathioprine - therapeutic use; Cholangitis - drug therapy; Cholangitis - pathology; Cholangitis - physiopathology; Female; Gastroenterology and Hepatology; Humans; Immunosuppressive Agents - therapeutic use; Male; Middle Aged; Pancreatitis - drug therapy; Pancreatitis - pathology; Pancreatitis - physiopathology; Prospective Studies; Recurrence; Steroids - therapeutic use; Treatment Failure; Treatment Outcome; AIH; computed tomography; PSC; AIP; AZA; autoimmune pancreatitis; azathioprine; IAC; IgG4-associated cholangitis; endoscopic retrograde cholangiopancreatography; CT; autoimmune hepatitis; MRCP; ERCP; magnetic resonance cholangiopancreatography; primary sclerosing cholangitis
• ISSN: 1542-3565; 1542-7714
• DOI: 10.1016/j.cgh.2009.03.021

Background & Aims Autoimmune pancreatitis (AIP) is a multisystem disorder that often has extrapancreatic manifestations such as immunoglobulin G4–associated cholangitis (IAC). Patients respond rapidly to steroids but can relapse after therapy. We assessed the clinical management of relapse in a group of patients with AIP/IAC. Methods We performed a prospective study of patients diagnosed with AIP from 2004–2007 who received steroids. Treatment outcome was defined clinically, radiologically, and biochemically as response to steroids, remission after steroids, failure to wean steroids, and relapse. Steroids ± azathioprine (AZA) were used to treat patients who failed, relapsed, or could not be weaned from steroids. Results Twenty-eight patients with AIP were studied; 23 (82%) had IAC. All patients responded within 6 weeks to prednisolone therapy. Twenty-three patients achieved remission after a median of 5 months of treatment (range, 1.5–17 months), whereas 5 patients (18%) could not be weaned because of a disease flare. Of the patients who achieved remission, 8 of 23 (35%) subsequently relapsed. Overall, 13 of 23 patients (57%) with AIP/IAC relapsed, compared with 0 of the 5 with isolated AIP ( P = .04, Fisher exact test). Steroids were increased/restarted in all patients who relapsed; 10 also received AZA. Remission was achieved and maintained in 7 patients; they remain on AZA monotherapy at a median of 14 months (range, 1–27 months). Conclusions Relapse or failure to wean steroids occurred in 46% of patients with AIP. Patients with IAC are at particularly high risk of relapse. AZA appears to be effective in patients with post-treatment relapse or who cannot be weaned from steroids. To view this article's video abstract, go to the AGA's YouTube Channel.