A case series in patients with enteropathy and granulomatous diseases

BMC Gastroenterology

Michael Schumann³  Severin Daum³  Britta Siegmund³  Imke Puls²  Verena Moos³  Korinna Jöhrens¹  Tassilo Kruis³ 

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

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

Others  :  1211527
DOI  :  10.1186/s12876-015-0292-4

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

【 摘 要 】

Background

Although 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 presentation

We 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.

Conclusions

Associations 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】

   
2015 Kruis et al.; licensee BioMed Central.

【 图 表 】

Fig. 1.

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