Familial autoimmune cholangiopathy and primary biliary cirrhosis.
نویسندگان
چکیده
منابع مشابه
Prevalence and pattern of familial disease in primary biliary cirrhosis.
Susceptibility to primary biliary cirrhosis (PBC) may be partly inherited although instances of PBC within families are only infrequently described. The records of 736 patients with PBC seen over a 25 year period were examined to identify those with a positive family history. Ten patients originating from eight families were identified, giving a frequency of 1.33%. They comprised mother and dau...
متن کاملPrimary biliary cirrhosis and Sjögren's syndrome: autoimmune epithelitis.
Primary biliary cirrhosis (PBC) has been often coined a model autoimmune disease based on the homogeneity amongst patients, the frequency and similarity of antimitochondrial antibodies, including the highly directed immune response to pyruvate dehydrogenase (PDC-E2). A significant number of patients with PBC suffer from sicca and amongst these, there are patients who also have classic Sjögren's...
متن کاملOverlap syndrome: autoimmune hepatitis with primary biliary cirrhosis.
"Overlap syndrome" is used to describe variant forms of autoimmune hepatitis (AIH) which present with characteristics of AIH and primary biliary cirrhosis (PBC) or primary sclerosing cholangitis (PSC). Patients with overlap syndromes present with both hepatitic and cholestatic serum liver tests and have histological features of AIH and PBC or PSC. AIH-PBC is the most common form of overlap synd...
متن کاملAnatomic Pathology / DISTINGUISHING PRIMARY BILIARY CIRRHOSIS FROM AUTOIMMUNE HEPATITIS
We studied nondiagnostic liver biopsy specimens from 20 patients with definite primary biliary cirrhosis (PBC) and 18 with definite autoimmune hepatitis (AIH) to identify distinguishing features. All patients had early-stage disease; biopsy specimens were devoid of granulomas or diagnostic features of PBC or AIH. Diagnoses were based on serologic and clinical variables. Sixteen specimens from e...
متن کاملPrimary Biliary Cirrhosis Is a Generalized Autoimmune Epithelitis
Primary biliary cirrhosis (PBC) is a chronic progressive autoimmune cholestatic liver disease characterized by highly specific antimitochondrial antibodies (AMAs) and the specific immune-mediated injury of small intrahepatic bile ducts. Unique apoptotic feature of biliary epithelial cells (BECs) may contribute to apotope presentation to the immune system, causing unique tissue damage in PBC. Pe...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Kanzo
سال: 1996
ISSN: 0451-4203,1881-3593
DOI: 10.2957/kanzo.37.336