Association of primary sclerosing cholangitis, ulcerative colitis and coeliac disease in female siblings
Autor: | Janina Orlowska, Eugeniusz Butruk, Aleksandra Lewartowska, Tomasz Rawa, Andrzej Habior, Jacek Pawlak, Andrzej Tilszer, Monika Sankowska, Marek Krawczyk |
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Rok vydání: | 2002 |
Předmět: |
Adult
medicine.medical_specialty Cirrhosis Cholangitis Sclerosing Disease Gastroenterology Coeliac disease Primary sclerosing cholangitis Liver disease HLA-DQ Antigens Immunopathology Internal medicine Genetic predisposition Humans Medicine Hepatology business.industry HLA-DR Antigens Middle Aged medicine.disease Ulcerative colitis digestive system diseases Celiac Disease Colitis Ulcerative Female business |
Zdroj: | European Journal of Gastroenterology & Hepatology. 14:787-791 |
ISSN: | 0954-691X |
Popis: | Primary sclerosing cholangitis is a rare, cholestatic liver disease, most commonly affecting young men. The association of primary sclerosing cholangitis with other autoimmune disorders, although rare, indicates a genetic predisposition for this disease. We describe, for the first time, the association of primary sclerosing cholangitis, ulcerative colitis and coeliac disease in two sisters. Ulcerative colitis was mild and preceded liver disease in both patients. There were no symptoms of coeliac disease, and its silent form was diagnosed on the basis of serological tests. Both patients carried HLA molecules DR3 and DQ2. Although HLA DR4 was not found, there was a rapid progression of liver disease to cirrhosis and cholangiocarcinoma in one patient. The familial occurrence of primary sclerosing cholangitis, ulcerative colitis and coeliac disease supports the hypothesis of genetic predisposition for these diseases. |
Databáze: | OpenAIRE |
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