A case series in patients with enteropathy and granulomatous diseases

BMC Gastroenterology

Case Report

Imke Puls¹  Korinna Jöhrens²  Britta Siegmund³  Tassilo Kruis³  Verena Moos³  Severin Daum³  Michael Schumann³ 

[1] Department for Psychiatry, Charité – Universitätsmedizin Berlin, Campus Mitte, Berlin, Germany;Institute for Pathology, Charité – Universitätsmedizin Berlin, Campus Mitte, Berlin, Germany;Medical Department I (Gastroenterology, Infectious Diseases, Rheumatology), Charité – Universitätsmedizin Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, 12200, Berlin, Germany;

关键词: Sarcoidosis;    Löfgren’s syndrome;    Celiac disease;    Autoimmune enteropathy;    AIE-75;    HLA-DR3/DQ2;   

DOI  :  10.1186/s12876-015-0292-4

 received in 2015-02-10, accepted in 2015-05-18,  发布年份 2015

来源: Springer

【 摘 要 】

BackgroundAlthough sarcoidosis and celiac disease are both chronic immunologic disorders involving multiple organ systems, reports about association of diseases in individual patients are sparse. While sarcoidosis is a chronic granulomatous disease presumably reflecting an exaggerated response to an unknown antigen, celiac disease is a T cell-driven disease triggered by ingestion of gluten, a protein composite found in wheat and related grains.Case presentationWe present three cases with a longstanding history of sarcoidosis that have been additionally diagnosed with celiac-like enteropathy. In two cases, celiac disease was established applying celiac-specific serology and duodenal histology, while one case was revealed as an AIE-75-positive autoimmune enteropathy.The HLA-DR3/DQ2 haplotype was confirmed in both celiac patients, hence confirming previous data of linkage disequilibrium as a cause for disease association. Remarkably, one celiac patient presented with granulomatous nodulae in the ileum, thus reflecting an intestinal sarcoid manifestation. In contrast the patient with an autoimmune enteropathy, was HLA-DQ9/DQ6-positive, also arguing against CD.ConclusionsAssociations of sarcoidosis and celiac disease are rare but do occur. Determining the HLA status in patients with complex autoimmune associations might help classifying involved disease entities.

【 授权许可】

Unknown   
© Kruis et al.; licensee BioMed Central. 2015. This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.