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A case series in patients with enteropathy and granulomatous diseases.

Kruis T, Jöhrens K, Moos V, Puls I, Siegmund B, Daum S, Schumann M - BMC Gastroenterol (2015)

Bottom Line: We present three cases with a longstanding history of sarcoidosis that have been additionally diagnosed with celiac-like enteropathy.In contrast the patient with an autoimmune enteropathy, was HLA-DQ9/DQ6-positive, also arguing against CD.Determining the HLA status in patients with complex autoimmune associations might help classifying involved disease entities.

View Article: PubMed Central - PubMed

Affiliation: Medical Department I (Gastroenterology, Infectious Diseases, Rheumatology), Charité - Universitätsmedizin Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, Berlin, 12200, Germany. tassilo.kruis@charite.de.

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.

No MeSH data available.


Related in: MedlinePlus

Ileal findings of patient C. a) Ileocolonoscopic follicular hyperplasia. b) Hematoxylin and eosin staining of ileal biopsies showing multinucleated macrophages (asterisks) and an epithelioid-cell granuloma (black arrow)
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Fig1: Ileal findings of patient C. a) Ileocolonoscopic follicular hyperplasia. b) Hematoxylin and eosin staining of ileal biopsies showing multinucleated macrophages (asterisks) and an epithelioid-cell granuloma (black arrow)

Mentions: Since childhood the 47-year-old Caucasian male was suffering from chronic abdominal discomfort, bloating, diarrhoea, and weight loss. At the age of 35 CD was suspected for the first time when duodenal biopsies revealed total villous atrophy and crypt hyperplasia. Gliadin-antibodies were highly positive. CD was also diagnosed in two of his brothers and in his niece. At the same time a CT-scan of the abdomen revealed iliac, paraaortic, and mesenteric lymphadenopathy as well as splenomegaly. Lymph node analysis showed epithelioid-cell granulomas without any histopathological evidence for mycobacteriosis. The patient started a GFD that led to partial improvement of his symptoms and normalization of duodenal mucosa as well as Tg-, gliadin-, and endomysium-antibodies. However, episodes of abdominal discomfort and diarrhoea remitted. Neither duodenal histology nor flow cytometric analysis of IEL indicated an abnormal IEL phenotype. Repeated colonoscopies performed between the ages of 41 to 46 years revealed a follicular hyperplasia of the terminal ileum without any evidence for mucosal ulcerations as typically found in Crohn’s disease (Fig. 1). Histology of one biopsy from the terminal ileum uncovered epithelioid-cell granulomas. A thorough work-up for an underlying infectious disease was inconclusive, except for positive antibodies against Yersinia spp. HLA-typing revealed the carriage of DR3/DQ2 (Table 2).Fig. 1


A case series in patients with enteropathy and granulomatous diseases.

Kruis T, Jöhrens K, Moos V, Puls I, Siegmund B, Daum S, Schumann M - BMC Gastroenterol (2015)

Ileal findings of patient C. a) Ileocolonoscopic follicular hyperplasia. b) Hematoxylin and eosin staining of ileal biopsies showing multinucleated macrophages (asterisks) and an epithelioid-cell granuloma (black arrow)
© Copyright Policy - open-access
Related In: Results  -  Collection

License 1 - License 2
Show All Figures
getmorefigures.php?uid=PMC4493942&req=5

Fig1: Ileal findings of patient C. a) Ileocolonoscopic follicular hyperplasia. b) Hematoxylin and eosin staining of ileal biopsies showing multinucleated macrophages (asterisks) and an epithelioid-cell granuloma (black arrow)
Mentions: Since childhood the 47-year-old Caucasian male was suffering from chronic abdominal discomfort, bloating, diarrhoea, and weight loss. At the age of 35 CD was suspected for the first time when duodenal biopsies revealed total villous atrophy and crypt hyperplasia. Gliadin-antibodies were highly positive. CD was also diagnosed in two of his brothers and in his niece. At the same time a CT-scan of the abdomen revealed iliac, paraaortic, and mesenteric lymphadenopathy as well as splenomegaly. Lymph node analysis showed epithelioid-cell granulomas without any histopathological evidence for mycobacteriosis. The patient started a GFD that led to partial improvement of his symptoms and normalization of duodenal mucosa as well as Tg-, gliadin-, and endomysium-antibodies. However, episodes of abdominal discomfort and diarrhoea remitted. Neither duodenal histology nor flow cytometric analysis of IEL indicated an abnormal IEL phenotype. Repeated colonoscopies performed between the ages of 41 to 46 years revealed a follicular hyperplasia of the terminal ileum without any evidence for mucosal ulcerations as typically found in Crohn’s disease (Fig. 1). Histology of one biopsy from the terminal ileum uncovered epithelioid-cell granulomas. A thorough work-up for an underlying infectious disease was inconclusive, except for positive antibodies against Yersinia spp. HLA-typing revealed the carriage of DR3/DQ2 (Table 2).Fig. 1

Bottom Line: We present three cases with a longstanding history of sarcoidosis that have been additionally diagnosed with celiac-like enteropathy.In contrast the patient with an autoimmune enteropathy, was HLA-DQ9/DQ6-positive, also arguing against CD.Determining the HLA status in patients with complex autoimmune associations might help classifying involved disease entities.

View Article: PubMed Central - PubMed

Affiliation: Medical Department I (Gastroenterology, Infectious Diseases, Rheumatology), Charité - Universitätsmedizin Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, Berlin, 12200, Germany. tassilo.kruis@charite.de.

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.

No MeSH data available.


Related in: MedlinePlus