Autoimmune pancreatitis in Hungary: a multicenter nationwide study

Abstract

Background: To date, most cases of autoimmune pancreatitis (AIP) have been reported from Japan. The aim of the present study was to assess the clinical features and management of AIP cases in Hungary.

Methods: The demographics, clinical presentation, laboratory and imaging findings, extrapancreatic involvement, treatment response and recurrence were evaluated in the first 17 patients diagnosed with AIP in Hungary.

Results: The mean age at presentation was 42.7 years (range: 16-74); 47% of the patients were women. New-onset mild abdominal pain (76%), weight loss (41%) and jaundice (41%) were the most common symptoms, with inflammatory bowel disease being the most frequent (36%) extrapancreatic manifestation. Diffuse pancreatic swelling was seen in 7 patients (41%) and a focal pancreatic mass in 8 (47%). Endoscopic retrograde cholangiopancreatography revealed pancreatic duct strictures in all study patients. The serum IgG4 level at presentation was elevated in 62% of the 8 patients in whom it was measured. All the percutaneous core biopsies (5 patients) and surgical specimens (2 patients), and 2 of the 4 biopsies of the papilla of Vater revealed the typical characteristic findings of AIP: a diffuse lymphoplasmacytic infiltration, marked interstitial fibrosis and obliterative phlebitis. Immunostaining indicated IgG4-positive plasma cells in 62% of the 8 patients in whom it was performed. Granulocytic epithelial lesions (GEL) were present in 3 patients. The patients without GELs were older (mean age 59 years), while those with GEL were younger (mean age 34 years), and 2 of 3 were female and had ulcerative colitis. A complete response to steroid treatment was achieved in all 15 patients. Because of the suspicion of a pancreatic tumor, 2 patients with focal AIP underwent partial pancreatectomy. One patient relapsed, but responded to azathioprine.

Conclusions: This first Hungarian series has confirmed several previously reported findings on AIP. AIP with GEL was relatively frequent among our patients: these patients tended to be younger than in earlier studies and displayed a female preponderance with a high coincidence of ulcerative colitis. Performance of a percutaneous biopsy is strongly recommended. The response to immunosuppressive therapy was excellent. and IAP.