BACKGROUND/AIMS: Autoimmune pancreatitis is a distinctive type of chronic pancreatitis with reversibility. We analyzed the largest number of patients with autoimmune pancreatitis among the studies carried out at a single institution in Korea. METHODS: We retrospectively analyzed the clinical, radiologic, laboratory and histologic features in 17 patients who were diagnosed as autoimmune pancreatitis. RESULTS: The patients were predominately elderly men who presented with jaundice or nonspecific gastrointestinal symptoms. In most of the patients, serum pancreatic enzymes were normal or mildly elevated with an accompanying cholestatic biochemical profile. Elevated IgG levels and detection of autoantibodies were observed in 47% (8/17) and 35% (6/17), respectively. Thirteen patients showed histologic findings of lymphoplasmacytic infiltration and fibrosis, and one patient showed predominant infiltration of eosinophils. Fifteen patients revealed radiologic images of diffuse swelling of pancreas and the sclerosing pattern on direct pancreatogram. All of the features improved in response to oral steroid therapy. Two cases showed focal narrowing with upstream duct dilatation and thus, they were diagnosed as focal type. Diabetes mellitus concurred in 13 cases and primary sclerosing cholangitis and Sjogren's syndrome were present in 1 case, respectively. CONCLUSIONS: The recognition of autoimmune pancreatitis allows patients to avoid unnecessary surgery and recover with the administration of oral steroid.
Aged ; Autoimmune Diseases/*diagnosis/pathology ; Chronic Disease ; English Abstract ; Humans ; Male ; Middle Aged ; Pancreas/pathology ; Pancreatitis/*diagnosis/immunology/pathology

Systemic amyloidosis is a disorder characterized by extracellular deposition of amyloid in various organs and tissues including the kidney, heart, and liver. However, pancreatic involvement is rare, and has not been reported in Korea. Systemic amyloisosis involving pancreas needs to be differentiated from several pancreatic diseases because of diffuse pancreatic enlargement and partial stricture or obstruction of main pancreatic duct. Recently, we experienced a 60-year old man who was suspected as autoimmune pancreatitis or infiltrative disorders on imaging studies, and finally diagnosed as systemic amyloidosis involving pancreas and liver on biopsy examination. We report the case with review of the relevant literatures.
Amyloidosis/*diagnosis/pathology/radiography ; Autoimmune Diseases/diagnosis ; Cholangiopancreatography, Endoscopic Retrograde ; Diagnosis, Differential ; Humans ; Male ; Middle Aged ; Pancreatic Diseases/*diagnosis ; Pancreatitis/diagnosis/immunology/pathology ; Tomography, X-Ray Computed

Autoimmune Diseases ; diagnosis ; immunology ; pathology ; surgery ; Humans ; Immunoglobulin G ; blood ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Pancreatectomy ; Pancreatitis ; diagnosis ; immunology ; pathology ; surgery

BACKGOUND/AIMS: The involvement of bile ducts is frequently reported in autoimmune pancreatitis (AIP), which seem to have similar features to primary sclerosing cholangitis (PSC). Recent systematic comparative studies about these diseases are rare in Korea. METHODS: We retrospectively analyzed 26 patients with AIP with bile duct involvement and 30 patients with classic PSC who were diagnosed during the last decade. RESULTS: The mean age of patients was significantly higher in AIP than PSC at the time of diagnosis. There was a preponderance of men in both group, which was more prominent in AIP. The most common symptom in patients with AIP was jaundice, but PSC patients usually visited hospitals due to incidentally detected abnormal liver function tests. Most (26/31) of AIP had bile duct involvement. All of these patients showed narrowing of intrapancreatic common bile ducts and one patient exhibited hilar involvement as well. About 80% of PSC had both intra- and extrahepatic ducts involvement, and the characteristic features involve multifocal strictures. AIP patients showed improvement with steroid treatment, however, most PSC patients showed clinical deterioration. CONCLUSIONS: The clinical and cholangiographic findings of patients with AIP and PSC have many different characteristics. Therefore, further study of two diseases is required for the proper diagnosis and management.
Autoimmune Diseases/*diagnosis/pathology/radiography ; Bile Ducts/pathology ; *Cholangiography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/*diagnosis/pathology/radiography ; Diagnosis, Differential ; Female ; Humans ; Immunoglobulins/blood ; Male ; Pancreatitis/*diagnosis/*immunology/pathology ; Retrospective Studies ; Sex Factors

OBJECTIVETo investigate clinicopathological features of fibrous mass-forming chronic pancreatitis (FMCP), to compare clinicopathological and immunohistochemical characteristics between autoimmune pancreatitis (AIP) and fibrous mass-forming non-autoimmune pancreatitis (nAIP) and to provide evidence for pathological diagnosis, differential diagnosis and clinical treatment strategy.

METHODSClinicopathological features were analyzed in 81 cases of FMCP. Infiltrating IgG4(+) plasmacytes were counted by immunohistochemical staining.

RESULTSAmong 81 cases of FMCP, 20 cases were diagnosed as AIP and 61 cases were interpreted as nAIP. AIP was more common in males over 50 years, whereas nAIP was seen in much younger patients (P = 0.001). The amount of inflammatory cells in the stroma of AIPs was remarkable higher than that in nAIPs (P = 0.002). The incidence of neuritis in AIPs (100%, 20/20) was also higher compared with that of nAIPs (75.4%, 46/61; P = 0.017). Storiformed-fibrosis was more common in AIPs (95.0%, 19/20) than in nAIPs (1.6%, 1/61;P = 0.000). Pancreatic intraepithelial neoplasia (PanIN) was observed in 50.0%(10/20) of AIPs and 32.8%(20/61) of nAIPs, with a greater severity observed in AIPs (P = 0.031). Tubular complex (TC) was more commonly observed in AIPs (65.0%, 13/20) than nAIPs (26.2%, 16/61;P = 0.002). Among 81 cases of FMCP, 61 cases had less than 11 IgG4(+) plasmacytes /HPF, 7 cases had 10-30/HPF and 13 cases had over 30/HPF.

CONCLUSIONSFMCPs include both AIP and nAIP. AIP has distinct pathological features and the presence of IgG4(+) plasmacyte is an important diagnostic parameter. FMCP appears to be an important precancerous lesion of pancreatic ductal adenocarcinoma. Surgery may be considered for patients with FMCP due to its mass-forming nature. In contrast, patients with AIP are treated medically due to its steroid-responsiveness. Therefore, accurate and timely diagnosis of AIP is of clinical relevance to avoid unnecessary surgical complications and to prevent progression of the disease.

Adult ; Aged ; Autoimmune Diseases ; immunology ; pathology ; surgery ; Carcinoma, Pancreatic Ductal ; immunology ; pathology ; surgery ; Diagnosis, Differential ; Female ; Fibrosis ; Humans ; Immunoglobulin G ; metabolism ; Male ; Middle Aged ; Pancreas ; pathology ; Pancreatic Neoplasms ; immunology ; pathology ; surgery ; Pancreatitis, Chronic ; immunology ; pathology ; surgery ; Plasma Cells ; immunology ; Precancerous Conditions ; immunology ; pathology ; surgery ; Young Adult

No abstract available.
Autoimmune Diseases/*diagnosis/drug therapy/immunology ; Azathioprine/therapeutic use ; Common Bile Duct/pathology ; Emphysema ; Fibrosis ; Humans ; Immunoglobulin G/blood ; Immunosuppressive Agents/therapeutic use ; Lung/*radiography ; Male ; Middle Aged ; Pancreatitis/*diagnosis/drug therapy/immunology ; Stents ; Tomography, X-Ray Computed