Presentation and management of post-treatment relapse in autoimmune pancreatitis/immunoglobulin G4-associated cholangitis.
OBJECTIVE
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.
Sandanayake
Neomal S
NS
Department of Gastroenterology, University College Hospital, London, United Kingdom; Institute of Hepatology, University College London, London NW1 2BU, United Kingdom.
Church
Nicholas I
NI
Chapman
Michael H
MH
Johnson
Gavin J
GJ
Dhar
Dipok K
DK
Amin
Zahir
Z
Deheragoda
Maesha G
MG
Novelli
Marco
M
Winstanley
Alison
A
Rodriguez-Justo
Manuel
M
Hatfield
Adrian R W
AR
Pereira
Stephen P
SP
Webster
George J M
GJ
D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000893:Anti-Inflammatory Agents / Q000627:therapeutic use*; D001327:Autoimmune Diseases / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology*; D001379:Azathioprine / Q000627:therapeutic use; D002761:Cholangitis / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology*; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D010195:Pancreatitis / Q000188:drug therapy* / Q000473:pathology / Q000503:physiopathology*; D011446:Prospective Studies; D012008:Recurrence; D013256:Steroids / Q000627:therapeutic use*; D017211:Treatment Failure; D016896:Treatment Outcome
D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D013256:Steroids; D001379:Azathioprine