Steroid treatment improves the exocrine and endocrine insufficiency, and the clinical and morphologic alterations in autoimmune pancreatitis

Background. Autoimmune pancreatitis (AIP) is a recently recognized clinical entity which accounts for approximately 5% of chronic pancreatitis. To the best of our knowledge, there has been only one report of AIP in Hungary; however, the exocrine and endocrine pancreatic function and the response to therapy were not studied. We describe a case of AIP in which the symptoms, morphologic alterations and exocrine and endocrine insufficiency regressed after a short course of oral steroid therapy.

Patient. A 62-year-old teetotal female presented with a 4-month history of recurrent epigastric pain and a 5-kg weight loss. There was no personal or family history of pancreatitis. An apple-sized, painless mass was palpated in the epigastrium. The fasting glucose level was increased and the faecal elastase test result was abnormal. The oral glucose tolerance test (OGTT) revealed diabetes mellitus. Antinuclear antibody and rheumatoid factor tests were positive. Ultrasonography (US) showed a diffusely enlarged pancreas, the CT scan revealed an enlarged sausage-shaped pancreas, and ERCP demonstrated an irregular main pancreatic duct with long strictures in the head and tail. The diagnosis was AIP. The patient was started on 32mg prednisolone daily and enzyme replacement therapy. After 4 weeks she was totally asymptomatic. The OGTT and faecal elastase test results were normal. The repeated US and CT scan revealed a marked improvement of the diffuse pancreatic swelling. On repeated ERCP, the main pancreatic duct narrowing was ameliorated. We advised tapering and cessation of the steroid.

Conclusion. It is important to be aware of this disease and its diagnosis, because AIP can clinically resemble as pancreatobiliary malignancies, or chronic or acute pancreatitis. However, in contrast with chronic pancreatitis, its symptoms and morphologic and laboratory alterations are completely reversed by oral steroid therapy.

Supported by ETT No. 5K 503 and BÖ No. 5/2003.