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Background. Autoimmune pancreatitis (AIP) is a relatively newly recognized type of pancreatitis that is characterized by diffuse or focal swelling of the pancreas due to lymphoplasmacytic infiltration and fibrosis of the pancreatic parenchyma. Material and methods. A PubMed literature search was performed using the keywords “autoimmune pancreatitis”. Sometimes, bibliographies were cross-referenced and related article searches were performed once an article of interest was identified. Results. Pathologically, AIP shows narrowing of the pancreatic ducts and the intrapancreatic portion of the common bile duct. Obstructive jaundice is a common symptom at presentation, and pancreatic cancer represents an important clinical differential diagnosis. In late stages of the disease, the normal pancreatic parenchyma is often replaced by large amounts of fibrosis. Histologically, there seem to be two subtypes of the disease–one showing infiltration with IgG4-positive plasma cells but lacking granulocytic epithelial lesions (GELs), the other showing GELs but lacking strong IgG4 positivity. AIP is in at least some instances the pancreatic manifestation of a clinicopathological entity of IgG4-related systemic sclerosing disease. On the basis of pancreatic imaging, together with serological measurement of IgG4 and evaluation of other organ involvement, many AIP patients can be identified. The remaining patients require further diagnostic work-up. In these patients, pancreatic core needle biopsy and, as AIP responds to steroid treatment, also a trial with steroids, can help to differentiate AIP from pancreatic cancer. Outlook and discussion. This review presents the pathological, radiologic and laboratory findings of AIP. Moreover, the treatment and pathogenesis are discussed. [ABSTRACT FROM AUTHOR]

Background: Although many patients with autoimmune pancreatitis undergo steroid therapy, detailed evaluation of morphological changes in the pancreas and bile duct following therapy has not been performed in this disease. In this study serological and morphological changes occurring during steroid treatment of autoimmune pancreatitis are comparatively examined. Methods: Ten patients with autoimmune pancreatitis were treated with corticosteroids. Morphological findings were: pancreatic enlargement ( n = 9), irregular narrowing of the main pancreatic duct ( n = 10), and biliary stenosis ( n = 9). An initial dose of prednisolone was 40-30 mg/day, and this was tapered by 5 mg every 1-2 weeks. All patients underwent ultrasound and serological testing 1-2 weeks after commencing medication, followed by weekly serological testing and by CT and endoscopic retrograde cholangiopancreatography after1-2 months. Radiological and serological changes were compared. Results: All 10 patients were responsive to steroid therapy. Pancreatic size normalized within 1 month; however, irregularity of the pancreatic duct remained in 6 patients. Rigidity or lateral deformity of the bile duct remained in 3 patients and biliary stenosis persisted in 5. Four patients in whom elevated serum IgG4 failed to normalize also showed incomplete morphological improvement. Three patients with complete improvement of the pancreatic duct stopped medication, but recurrence of pancreatitis did not occur. Conclusions: Although steroid therapy was morphologically and serologically effective in patients with autoimmune pancreatitis, cholangiopancreatographic abnormalities remained in many patients. Morphological improvement on cholangiopancreatography and normalization of serum IgG4 after steroid therapy appeared to be good indicators for discontinuing medication in patients with autoimmune pancreatitis. [ABSTRACT FROM AUTHOR]