Autoimmune pancreatitis in Hungary. A multicenter nationwide study

Background: 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 assessed in the first 10 patients diagnosed with AIP in Hungary. Results: The median age at presentation was 44 years (range: 19–74); 50% were men. New-onset mild abdominal pain (90%), weight loss (50%) and jaundice (30%) were the most common symptoms, with biliary strictures and ulcerative colitis as the most frequent (30%) extrapancreatic manifestations. Diffuse pancreatic swelling was seen in 7 patients (70%), and a focal mass in 3 (30%). Pancreatic duct strictures were present in all 10 patients. The serum immunoglobulin-G4 level was elevated in 66% at presentation. All the percutaneous core biopsies (3 patients) and surgical specimens (2 patients) and in 2 out of 4 cases the biopsy of the papilla of Vater revealed the typical characteristic findings of AIP: diffuse lymphoplasmacytic infiltration, marked interstitial fibrosis, and obliterative phlebitis. A complete response to steroid treatment was achieved in all 10 patients. Because of the suspicion of pancreatic tumor, 2 patients with focal AIP underwent partial pancreatectomy, Recurrences were not observed. The Japanese Pancreas Society diagnostic criteria for AIP were fulfilled in 80% of these cases. Conclusions: In this first Hungarian series, we have confirmed several findings previously reported in AIP. The occurrence in young patients and the lack of a male preponderance were interesting features of AIP in this series. The response to immunosuppressive therapy was excellent. The performance of percutaneous biopsy is highly recommended.