A case series in patients with enteropathy and granulomatous diseases.

Abstract

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.

• Authors: Tassilo Kruis, Korinna Jöhrens, Verena Moos, Imke Puls, Britta Siegmund, Severin Daum, Michael Schumann
• Journal: BMC gastroenterology (BMC Gastroenterol) Vol. 15 Pg. 62 (May 23 2015) ISSN: 1471-230X [Electronic] England
• PMID: 26001889 (Publication Type: Case Reports, Journal Article)

Chemical References

• Biomarkers
• HLA-DQ Antigens
• HLA-DQ2 antigen
• HLA-DQ6 antigen
• HLA-DQ9 antigen

Topics

• Adult
• Biomarkers (blood)
• Celiac Disease (blood, complications, diagnosis, immunology)
• Diagnosis, Differential
• Female
• HLA-DQ Antigens (blood)
• Haplotypes
• Humans
• Lung Diseases (blood, complications, diagnosis, immunology)
• Middle Aged
• Polyendocrinopathies, Autoimmune (blood, complications, diagnosis, immunology)
• Sarcoidosis (blood, complications, diagnosis, immunology)