Humans ; Liver Cirrhosis, Biliary ; pathology ; surgery ; Liver Transplantation ; Recurrence

No abstract available.
Female ; Humans ; Liver Cirrhosis, Biliary/*diagnosis/pathology/radiography ; Middle Aged

Autoimmune cholangitis is a clinical constellation of chronic cholestasis, histological changes of chronic nonsuppurative cholangitis and the presence of autoantibodies other than antimitochondrial antibody (AMA). It is uncertain whether this entity is definitely different from AMA positive primary biliary cirrhosis (PBC), though it shows some differences. We report a case of autoimmune cholangitis in a 59-year-old woman, who had been previously diagnosed as AMA-positive PBC associated with rheumatoid arthritis, has been converted to an AMA-negative and anticentromere antibody-positive PBC during follow-up. The response to ursodeoxycholic acid treatment is poor except within the first few months, but prednisolone was dropping the biochemical laboratory data.
Autoantibodies/immunology* ; Case Report ; Cholangitis/pathology ; Cholangitis/immunology* ; Female ; Human ; Liver Cirrhosis, Biliary/pathology ; Liver Cirrhosis, Biliary/immunology* ; Middle Age ; Mitochondria/immunology*

No abstract available.
Adult ; Bile Ducts/pathology ; Female ; Humans ; Liver Cirrhosis, Biliary/*genetics/pathology ; Middle Aged

No abstract available.
Adult ; Cholangitis, Sclerosing/*diagnosis/pathology ; Diagnosis, Differential ; Humans ; Liver Cirrhosis, Biliary/diagnosis ; Male

Adult ; Antigens, Differentiation, T-Lymphocyte ; blood ; Female ; Humans ; Liver Cirrhosis, Biliary ; blood ; pathology ; Male ; Middle Aged

Nodular regenerative hyperplasia (NRH) of the liver is a rare disease that is characterized by multiple regenerative nodules in the hepatic parenchyma without fibrosis. The exact pathogenesis of NRH has not been established, but it's been suggested that obliteration of portal veins may initiate the nodular transformation. It is also known that this disease is associated with autoimmune disease, myeloproliferative disease, lymphoproliferative disease, primary biliary cirrhosis, and some chemotherapy agents. The patients with NRH are usually asymptomatic, yet if they have symptoms, the most common clinical manifestations are those of portal hypertension, including splenomegaly and esophageal varices with or without bleeding. We report a case of nodular regenerative hyperplasia that presented with clinical manifestations similar to those of primary biliary cirrhosis.
Aged ; Diagnosis, Differential ; English Abstract ; Female ; Focal Nodular Hyperplasia/*diagnosis/pathology ; Humans ; Hyperplasia ; Liver/*pathology ; Liver Cirrhosis, Biliary/*diagnosis

OBJECTIVEClinical and liver pathological features of 60 primary biliary cirrhosis (PBC) patients were reviewed to identify prognostic factors in order to improve the diagnosis and treatment of the disease.

METHODSThe general conditions, clinical manifestations, serum biochemical and immunological changes, and liver pathological findings were assessed in 60 PBC patients. All cases were followed up and 5 variables were studied by univariate analysis; the variables linked with survival were included in a Cox model.

RESULTSForty-eight patients were females (80%), 12 were males (20%), and the mean age at their diagnoses was (52.5+/-9.4). The symptoms most frequently complained about were jaundice (61.6%), fatigue (51.6%), anorexia (43.3%) and pruritus (25%). Serum alkaline phosphatase (ALP) and glutamyl transpeptidase (GGT) levels were markedly elevated in the majority of the patients [(242.3+/-137.1) U/L and (250.6+/-216.1) U/L, respectively], whereas ALT and AST levels were mildly to moderately elevated [(185.8+/-269.1) U/L and (172.5+/-163.6) U/L, respectively]. Thirty-two patients (53.3%) had a total bilirubin level of > or = 34.2 micromol/L. Twenty-eight patients (59.5%) had elevated serum IgM and 41 patients (68.3%) were anti-mitochondrial antibody AMA/AMA-M2 positive. Forty-two of the 60 patients had liver biopsies. The liver pathological changes: 33.3% of the cases were in I or II stage and 66.6% in III or IV stage. The follow up results: Five patients died of liver failure or massive upper gastrointestinal bleeding; 45 were still alive; the average survival period was 3.92 years; 10 patients were lost in the follow-up. With multivariate analysis (Cox model), age, level of total bilirubin and the stage of the liver pathological changes were found to be independent factors linked to the survival of the patients.

CONCLUSIONPBC may not be a rare liver disease in China. The awareness to recognize PBC is important in making an early diagnosis and treatment.

Adult ; Aged ; Aged, 80 and over ; Female ; Humans ; Liver ; pathology ; Liver Cirrhosis, Biliary ; diagnosis ; pathology ; Male ; Middle Aged ; Prognosis ; Young Adult

Adult ; Female ; Humans ; Liver ; immunology ; pathology ; Liver Cirrhosis, Biliary ; immunology ; pathology ; Male ; Middle Aged ; T-Lymphocytes, Regulatory ; immunology

Sjogren's syndrome (SS) is an autoimmune disease characterized by a lymphocytic infiltration of the salivary and lacrimal glands leading to a progressive destruction of these glands due to the production of autoantibodies. This disorder is either isolated (primary SS) or associated with other systemic diseases (secondary SS). The occurrence of B-cell non-Hodgkin's lymphoma (NHL) represents the major complication in the evolution of SS patients. The risk of developing NHL, which is equivalent for both primary and secondary SS, was estimated to be 44 times greater than that observed in a comparable normal population. NHLs in SS patients occur preferentially in the salivary glands and in other mucosa-associated lymphoid tissues (MALT). However, it can also occur in the lymph nodes or bone marrow. We documented a case of low-grade B-cell lymphoma of MALT in the right eyelid and primary biliary cirrhosis (PBC) of a patient with SS. To the best of our knowledge, this is the first case reported in Korea.
Case Report ; Eyelid Neoplasms/pathology ; Eyelid Neoplasms/etiology* ; Female ; Human ; Liver Cirrhosis, Biliary/pathology ; Liver Cirrhosis, Biliary/complications* ; Lymphoma, Mucosa-Associated Lymphoid Tissue/pathology ; Lymphoma, Mucosa-Associated Lymphoid Tissue/etiology* ; Middle Age ; Sjogren's Syndrome/pathology ; Sjogren's Syndrome/complications*