Autoantibodies ; blood ; Cholangitis, Sclerosing ; complications ; diagnosis ; Hepatitis, Autoimmune ; complications ; diagnosis ; Humans ; Liver Cirrhosis, Biliary ; complications ; diagnosis

IgG4-associated cholangitis can mimic hilar cholangiocarcinoma. Previously reported patients with IgG4-associated cholangitis mimicking cholangiocarcinoma had elevated serum IgG4 levels and long-segment biliary strictures. However, in the absence of other diagnostic criteria for malignancy, IgG4-associated cholangitis should remain a consideration among patients with normal serum IgG4 and a hilar mass suspicious for cholangiocarcinoma. The presence of a hilar mass and a malignant-appearing biliary stricture in two patients with normal serum IgG4 prompted further evaluation and subsequent concomitant liver and bile duct resection and reconstruction. The diagnosis of IgG4-associated cholangitis was established during the pathologic evaluation of the resected specimens. IgG4-associated cholangitis is a known imitator of hilar cholangiocarcinoma and should be considered in the differential diagnosis even among serologically IgG4-negative patients with a hilar mass prior to operative resection.
Aged ; Bile Ducts/pathology/surgery ; Cholangitis/blood/*diagnosis ; Diagnosis, Differential ; Humans ; Immunoglobulin G/*blood ; Klatskin Tumor/blood/*diagnosis ; Liver/pathology/surgery ; Male

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 the positive rate of auto antibodies and autoimmune liver diseases in patients with abnormal liver function and it's clinical significance.

METHODS511 sera with abnormal ALT (>40 U/L) were continuously collected, all the sera were examined for antibodies and clinical information of 469 cases were studied.

RESULTSAmong the 511 sera, 14.09% of them showed of ANA positive, 0.59% of SMA positive, 2.94% of AMA positive, 0.98% of AMA-M2 positive, 0.59% of SS-A positive, 0.19% of SS-B positive, 0.19% of JO-1 and 0.78% of dsDNA positive and all SLA/LP, LC-1 and LKM-1 and ANA profile were negative. Clinical information was analyzed on 469 cases which have complete data from the 511 patients. Of these 469 cases, 5 cases (1.06%) were found to be PBC, 2 case (0.43%) were AIH, no PSC was found, 77.78% patients among those with positive auto antibodies were diagnosed as viral hepatitis and there were 18.29% patients with viral hepatitis showed different auto antibodies.

CONCLUSIONThe high titer auto antibodies were important criterion for diagnosis of autoimmune liver diseases. The positive rate of autoantibodies of autoimmune liver diseases was similar to hepatitis C and E

Adolescent ; Adult ; Aged ; Aged, 80 and over ; Autoantibodies ; blood ; Child ; Child, Preschool ; Cholangitis, Sclerosing ; diagnosis ; immunology ; Diagnosis, Differential ; Female ; Hepatitis, Autoimmune ; diagnosis ; immunology ; Humans ; Liver ; physiopathology ; Liver Cirrhosis, Biliary ; diagnosis ; immunology ; Liver Function Tests ; Male ; Middle Aged

IgG4-related systemic diseases are characterized by a diffuse or mass forming inflammatory reaction rich in lymphocytes and IgG4-positive plasma cells (lymphoplasmacytic infiltration), fibrosclerosis of variable organs and obliterative phlebitis. They usually involve various organs including the pancreas, bile duct, gallbladder, salivary gland, retroperitoneum, kidney, lung, and prostate. However, most of them are accompanied by autoimmune pancreatitis, and good response to steroid treatment is one of the hallmarks of this disease. We report a case of an 67-year-old man with IgG4 associated sclerosing cholangitis, who was diagnosed by endoscopic retrograde cholangiopancreatography and successfully treated with steroid therapy.
Aged ; Anti-Inflammatory Agents/therapeutic use ; Autoimmune Diseases/complications/diagnosis ; Bile Ducts, Intrahepatic/pathology/ultrasonography ; Cholangiopancreatography, Endoscopic Retrograde ; Cholangitis, Sclerosing/complications/*diagnosis/drug therapy ; Common Bile Duct/pathology/ultrasonography ; Humans ; Immunoglobulin G/*blood ; Immunohistochemistry ; Male ; Pancreatitis/complications/diagnosis ; Prednisolone/therapeutic use ; Tomography, X-Ray Computed