Synonyms
Coeliac sprue; Gluten-induced enteropathy or gluten-sensitive enteropathy (GSE); Nontropical sprue
Definition
Coeliac disease (CD) is a chronic inflammatory disorder of the small intestine characterized by malabsorption after ingestion of wheat gluten or related proteins in rye (secalins) and barley (hordeins) in individuals with a certain genetic background. The pathogenesis involves a T cell-mediated immune response and autoreactive B lymphocytes that produce autoantibodies directed against gliadin, endomysium, or tissue transglutaminase in individuals with a genetic susceptibility related to HLA-DQ2 and HLA-DQ8. The diagnostic scheme of GSE consists of clinical history and symptomatology, HLA subtyping, serologic testing (tTG, EMA, and AGA), histological findings in proximal small intestinal biopsy, and clinical and serological (optionally histological) response to a GFD.
Clinical Features
Incidence
Current estimates show that the incidence is at 0.5–1% in wheat-eating...
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References and Further Reading
Corazza, G. R., & Villanaci, V. (2005). Coeliac disease. Journal of Clinical Pathology, 58, 573–574.
Dickson, B. C., Streutker, C. J., & Chetty, R. (2006). Coeliac disease: An update for pathologists. Journal of Clinical Pathology, 59, 1008–1016.
Ensari, A. (2010). Gluten-sensitive enteropathy (celiac disease): Controversies in diagnosis and classification. Archives of Pathology & Laboratory Medicine, 134, 826–836.
Kurppa, K., Ashorn, M., Iltanen, S., Koskinen, L. L. E., Saavalainen, P., Koskinen, O., Maki, M., & Kaukinen, K. (2010). Celiac disease without villous atrophy in children: a prospective study. The Journal of Pediatrics, 157, 373–380.
Marsh, M. N. (1992). Gluten, major histocompatibility complex and the small intestine: A molecular and immunobiologic approach to the spectrum of gluten sensitivity (“coeliac sprue”). Gastroenterology, 102, 330–354.
Marsh, M. N., & Crowe, P. T. (1995). Morphology of the mucosal lesion in gluten sensitivity. Baillière’s Clinical Gastroenterology, 9, 273–293.
Oberhuber, G., Granditsch, G., & Vogelsang, H. (1999). The histopathology of coeliac disease: Time for a standardized report scheme for pathologists. European Journal of Gastroenterology and Hepatology, 11, 1185–1194.
Özakıncı H, Kırmızı A, Savaş B, Kalkan Ç, Soykan İ, Çetinkaya H, Kuloğlu Z, Kansu A, Gürkan ÖE, Dalgıç B, Şentürk Z, Ensari A. (2016). Classification Chaos in coeliac disease: does it really matter? Pathol Res Pract. 212(12), 1174–1178.
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Ensari, A. (2017). Coeliac Disease. In: Carneiro, F., Chaves, P., Ensari, A. (eds) Pathology of the Gastrointestinal Tract. Encyclopedia of Pathology. Springer, Cham. https://doi.org/10.1007/978-3-319-40560-5_1432
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DOI: https://doi.org/10.1007/978-3-319-40560-5_1432
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