Narrative Review: Celiac Disease: Understanding a Complex Autoimmune Disorder

Abstract

Celiac disease is a common autoimmune disorder that has genetic, environmental, and immunologic components. It is characterized by an immune response to ingested wheat gluten and related proteins of rye and barley that leads to inflammation, villous atrophy, and crypt hyperplasia in the intestine. The disease is closely associated with genes that code for human leukocyte antigens DQ2 and DQ8. Transglutaminase 2 appears to be an important component of the disease, both as a deamidating enzyme that can enhance the immunostimulatory effect of gluten and as a target autoantigen in the immune response. Sensitive and specific serologic tests, including those for anti-transglutaminase antibody, are facilitating fast and noninvasive screening for celiac disease. Thus, they are contributing to a more accurate estimate of the prevalence of the disease and its association with other disorders. Celiac disease is associated with increased rates of anemia, osteoporosis, cancer, neurologic deficits, and additional autoimmune disorders. A gluten-free diet is the mainstay of safe and effective treatment of celiac disease, although its effect on some of the extraintestinal manifestations of the disease remains to be determined.

Celiac disease in the Red Sea state of Sudan

“…1,2,13 In this study, chronic diarrhea was the most common presenting symptom (20.3%). In Iran, Lebanon, Iraq, Saudi Arabia and Kuwait, CD has been reported to be one of the most common causes of chronic diarrhea.…”

Pharmacologic transglutaminase inhibition attenuates drug‐primed liver hypertrophy but not Mallory body formation

“…TG-generated crosslinks can be beneficial as occurs during blood clotting or formation of the epidermal cell-envelope, but can be pathologic since they may lead to autoimmune disorders such as celiac disease [3]. Within the TG family, TG2 (also known as tissue transglutaminase) is ubiquitously expressed and implicated in apoptosis, organization of extracellular matrix (ECM) and signal transduction [1,2].…”

Celiac disease diagnosis and gluten-free food analytical control

“…While two different types of tests are used for detect these autoantibodies, they detect antibodies to the same antigen, the tTG [56]. IgA anti-tTG antibody and IgA anti-EMA tests have sensitivities higher than 90% and specificities higher than 95% and, therefore, the serological tests to detect those antibodies are recommended for initial screening [57].…”

“…A biopsy might also be recommended in cases of negative serology, when there is a high clinical suspicion. In positive serological cases, followed by a negative biopsy, it is important to consider HLA typing, since the absence of both HLA-DQ2 and/or HLA-DQ8 alleles has a very high negative predictive value, helping to rule out the disease in cases of equivocal biopsy results [3,4,56].…”