Association of primary sclerosing cholangitis, ulcerative colitis and coeliac disease in female siblings.
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.
Habior
Andrzej
A
Department of Gastroenterology, Medical Centre for Postgraduate Education, Institute of Oncology, W.K. Roentgena 5, 02-781 Warsaw, Poland.
Rawa
Tomasz
T
Orłowska
Janina
J
Sankowska
Monika
M
Lewartowska
Aleksandra
A
Tilszer
Andrzej
A
Pawlak
Jacek
J
Krawczyk
Marek
M
Butruk
Eugeniusz
E
D000328:Adult; D002446:Celiac Disease / Q000150:complications / Q000235:genetics* / Q000276:immunology; D015209:Cholangitis, Sclerosing / Q000150:complications / Q000235:genetics* / Q000276:immunology; D003093:Colitis, Ulcerative / Q000150:complications / Q000235:genetics* / Q000276:immunology; D005260:Female; D006683:HLA-DQ Antigens / Q000032:analysis; D006684:HLA-DR Antigens / Q000032:analysis; D006801:Humans; D008875:Middle Aged
D006683:HLA-DQ Antigens; D006684:HLA-DR Antigens