Original articles—liver, pancreas, and biliary tract
Presentation and Management of Post-treatment Relapse in Autoimmune Pancreatitis/Immunoglobulin G4-Associated Cholangitis

https://doi.org/10.1016/j.cgh.2009.03.021Get rights and content

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.

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Section snippets

Patients and Methods

This was a prospective case series and included all patients who were diagnosed with AIP in our center between February 2004 and December 2007 and who received a course of steroid therapy. The diagnosis was based on recognized international criteria, including those from the Japan Pancreas Society12 and, since 2006, the HISORt criteria from the Mayo Clinic.4 Features included a focal pancreatic mass or a diffusely enlarged pancreas (characteristically termed sausage pancreas); focal or diffuse

Patient Profile and Clinical Disease at Diagnosis

AIP was diagnosed in 28 patients (M:F, 24:4) with median age at diagnosis of 61 years (range, 27–81 years) (Table 1). The initial presentation included obstructive jaundice in 23 of 28 patients (82%), whereas 4 (14%) presented with abdominal/back pain alone. In 1 patient an incidental pancreatic mass was noted on follow-up imaging for an abdominal aortic aneurysm. Five patients (18%) had undergone surgery after their initial presentation (before the diagnosis of AIP) in view of the suspicion of

Discussion

It is now generally accepted that AIP represents one aspect of a multisystem disease characterized by the involvement of affected tissues by an IgG4-positive plasma cell infiltrate.13, 14, 15 The term IgG4-related sclerosing disease16 has also been advocated, and IAC has been used to describe the biliary disease with which AIP is frequently associated.10

Although spontaneous improvement of pancreatic masses and biliary strictures can occur,3, 17, 18 a rapid response to steroid therapy is often

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    Conflicts of interest The authors disclose no conflicts.

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