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Coeliac disease

Nature Reviews Disease Primers volume 5, Article number: 3 (2019) Cite this article

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Abstract

Coeliac disease is an immune-mediated enteropathy against dietary gluten present in wheat, rye and barley and is one of the most common lifelong food-related disorders worldwide. Coeliac disease is also considered to be a systemic disorder characterized by a variable combination of gluten-related signs and symptoms and disease-specific antibodies in addition to enteropathy. The ingestion of gluten leads to the generation of harmful gluten peptides, which, in predisposed individuals, can induce adaptive and innate immune responses. The clinical presentation is extremely variable; patients may have severe gastrointestinal symptoms and malabsorption, extraintestinal symptoms or have no symptoms at all. Owing to the multifaceted clinical presentation, diagnosis remains a challenge and coeliac disease is heavily underdiagnosed. The diagnosis of coeliac disease is achieved by combining coeliac disease serology and small intestinal mucosal histology during a gluten-containing diet. Currently, the only effective treatment for coeliac disease is a lifelong strict gluten-free diet; however, the diet is restrictive and gluten is difficult to avoid. Optimizing diagnosis and care in coeliac disease requires continuous research and education of both patients and health-care professionals.

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Fig. 1: The global seroprevalence of coeliac disease.
Fig. 2: Cereals harmful for patients with coeliac disease.
Fig. 3: Gluten peptide presentation by HLA-DQ2.
Fig. 4: Adaptive immune responses involved in coeliac disease.
Fig. 5: Innate immune responses involved in coeliac disease.
Fig. 6: The clinical manifestations of coeliac disease.
Fig. 7: The continuum of small intestinal mucosal damage in coeliac disease.

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Acknowledgements

The authors thank for the Academy of Finland, the Sigrid Juselius Foundation and the Competitive State Research Financing of the Expert Area of Tampere University Hospital (K. Kaukinen, K.L. and K. Kurppa); the European Commission (FP6-FP7); Stichting Coeliakie Onderzoek Nederland; the European Society for Paediatric Gastroenterology, Hepatology and Nutrition (ESPGHAN; M.L.M.); the Indian government and non-government organizations (G.K.M.), the US NIH (J.A.M.); the Canadian Institutes of Health Research and Crohn’s Colitis Canada grants; the Nestle Research Center; and Biocodex (E.F.V.) for support and funding. J.A.M. also acknowledges philanthropic support from the Mayo Foundation.

Reviewer information

Nature Reviews Disease Primers thanks C. Catassi, T. Not and the other anonymous referee(s) for the peer review of this work.

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Authors and Affiliations

  1. Celiac Disease Research Center, Faculty of Medicine and Life Sciences, University of Tampere, Tampere, Finland

    Katri Lindfors

  2. Coeliac Center at Department of Medicine and Surgery, Scuola Medica Salernitana, University of Salerno, Salerno, Italy

    Carolina Ciacci

  3. Tampere Center for Child Health, University of Tampere and Tampere University Hospital, Tampere, Finland

    Kalle Kurppa

  4. Institute of Clinical Medicine and K.G. Jebsen Coeliac Disease Research Centre, Faculty of Medicine, University of Oslo, and Department of Gastroenterology, Oslo University Hospital, Oslo, Norway

    Knut E. A. Lundin

  5. Department of Gastroenterology and Human Nutrition, All India Institute of Medical Sciences, New Delhi, India

    Govind K. Makharia

  6. Department of Pediatrics, Leiden University Medical Center, Leiden, Netherlands

    M. Luisa Mearin

  7. The Mayo Clinic, Rochester, MN, USA

    Joseph A. Murray

  8. Division of Gastroenterology, Department of Medicine, Farncombe Family Digestive Health Research Institute, McMaster University, Hamilton, Ontario, Canada

    Elena F. Verdu

  9. Department of Internal Medicine, Tampere University Hospital and Faculty of Medicine and Life Sciences, University of Tampere, Tampere, Finland

    Katri Kaukinen

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Contributions

Introduction (K. Kaukinen and K.L.); Epidemiology (K. Kaukinen, K.L., G.K.M. and J.A.M.); Mechanisms/pathophysiology (K. Kaukinen, K.L. and E.F.V.); Diagnosis, screening and prevention (K. Kaukinen, K.L. and M.L.M.); Management (K. Kaukinen, K.L., K. Kurppa and K.E.A.L.); Quality of life (K. Kaukinen, K.L. and C.C.); Outlook (K. Kaukinen, K.L., C.C., K. Kurppa, K.E.A.L., G.K.M., M.L.M., J.A.M. and E.F.V.); Overview of the Primer (K. Kaukinen).

Corresponding author

Correspondence to Katri Kaukinen.

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Competing interests

None of the authors declares any financial competing interests. The authors have the following non-financial competing interests. K. Kaukinen, K.L. and K. Kurppa are members of the Scientific Advisory Board of the Finnish Coeliac Society. K. Kaukinen and K. Kurppa are members of the Finnish Coeliac Disease Current Care Guidelines committee. K. Kaukinen is a vice chairman of the Finnish Society of Internal Medicine. G.K.M. holds the post of Secretary General of the Indian Society of Gastroenterology, is a board member of the International Society for Studies on Coeliac Disease, is Co-Chair of the Research Committee of the World Gastroenterology Organization, serves as Coordinator of the Indian National Taskforce on Inflammatory Bowel Disease and is co-inventor of a device for faecal incontinence. J.A.M. is Section Editor for Mayo Clinic Proceedings. E.F.V. holds a Canada Research Chair and is an advisory board member of Innovate Pharmaceuticals, is President of the Society for the Study of Coeliac Disease, is Treasurer of the Canadian Association of Gastroenterology (CAG) and is an executive board member of CAG and the Canadian Digestive Health Foundation.

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Lindfors, K., Ciacci, C., Kurppa, K. et al. Coeliac disease. Nat Rev Dis Primers 5, 3 (2019). https://doi.org/10.1038/s41572-018-0054-z

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