Ulcerative colitis associated with IgG4 cholangitis: Similar features in two HLA identical siblings

JOURNAL OF HEPATOLOGY

卷:51

Article

Dastis, Sergio Negrin¹  Latinne, Dominique²  Sempoux, Christine³  Geubel, Andre P.¹ 

[1] Univ Catholique Louvain, St Luc Univ Hosp, Dept Gastroenterol & Hepatol, B-1200 Brussels, Belgium

[2] Univ Catholique Louvain, St Luc Univ Hosp, Dept Immunol, B-1200 Brussels, Belgium

[3] Univ Catholique Louvain, St Luc Univ Hosp, Dept Pathol, B-1200 Brussels, Belgium

关键词: Autoimmune cholangitis;    Ulcerative colitis;    IgG4;    HLA-identical siblings;   

DOI  :  10.1016/j.jhep.2009.05.032

来源: Elsevier

【 摘 要 】

Background. IgG4-associated cholangitis (IAC) can mimic primary sclerosing cholangitis although, in contrast to the latter, it is highly responsive to steroid therapy. IAC is known to be associated with autoimmune pancreatitis and has also been shown to be part of a more complex autoimmune IgG4 syndrome. However, an association with inflammatory bowel disease (IBD), a condition in which its identification may have therapeutic and prognostic importance, has not yet been described. Case reports: We report the cases of two H LA identical siblings both DRB*1501 positive exhibiting features of IAC together with ulcerative colitis. Subsequent high resolution HLA typing performed by sequence-based-typing showed similar alleles in both siblings: A*0301 A*3201 B*07 (0702/62) B*1401 C*0702 C*0802 DRB1*1501. There is indirect evidence that this hitherto undescribed association, likely to be strongly linked to a genetic background, might account for a proportion of the cases of cholangitis associated with IBD. Conclusion: Appropriate investigation for I BD-associated cholangitis is mandatory to identify IAC, the recognition of which has particular therapeutic and prognostic implications. (C) 2009 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

【 授权许可】

Free