Lege Artis Medicinae

[Autoimmune pancreatitis in Hungary: a national multicenter study]

CZAKÓ László, GYÖKERES Tibor, TAKÁCS Tamás, TOPA Lajos, SAHIN Péter, DUBRAVCSIK Zsolt, SZEPES Attila, PAP Ákos, FÖLDESI Imre, TISZLAVICZ László, WITTMANN Tibor

JANUARY 20, 2011

Lege Artis Medicinae - 2011;21(01)

[BACKGROUND - Autoimmune pancreatitis (AIP) is an increasingly recognised, special form of chronic pancreatitis, which greatly differs from other forms of chronic pancreatitis. Most papers on this condition have been published in Japan. METHODS - In our multicenter study, we aim to present the characteristics (demographics, clinical symptoms, laboratory and morphological findings, extrapancreatic symptoms, response to therapy, remission) of the first 13 Hungarian cases of AIP. RESULTS - The mean age at presentation was 44.2 years (range: 19-74); 54% of patients were women. New-onset mild abdominal pain (77%), weight loss (38%) and jaundice (31%) were the most common symptoms, with ulcerative colitis as the most frequent (38%) extrapancreatic manifestation. Diffuse pancreatic swelling was seen in 7 patients (54%), and a focal mass in 5 (38%). Pancreatic duct strictures were present in all patients. Serum immunoglobulin-G4 level was elevated in 71% of the patients in whom it was measured. All percutaneous core biopsies (4 patients) and surgical specimens (2 patients), and 2 of the 4 biopsies of the papilla of Vater revealed hystological findings typical for AIP: periductal, diffuse lymphoplasmacytic infiltration, marked interstitial fibrosis, and obliterative phlebitis. Immunostaining revealed IgG4-positive plasma cells in 57% of the patients in whom it was examined. Granulocytic epithelial lesions (GEL) were detected in 3 patients. These patients were younger (mean age 34 years), 66% were women and 6% had ulcerative colitis, whereas the mean age of patients without GEL was 65 years, and the majority of them were men. Steroid treatment resulted in remission of the symptoms in all patients. Because of suspicion of pancreatic tumour, 2 patients with focal AIP underwent partial pancreatectomy. Symptoms relapsed in one patient, but an increased dose of steroid resulted in a remission, which was maintained by azathioprine therapy. CONCLUSIONS - In our first Hungarian cases, we have confirmed previously reported characteristics of AIP. AIP with GEL is frequent among our patients; this condition is more frequent among women and younger patients, and is often associated with ulcerative colitis. If AIP is suspected, the performance of percutaneous biopsy is highly recommended. The therapeutic response to steroid therapy was excellent.]

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