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Extraintestinal manifestations of coeliac disease

Nature Reviews Gastroenterology & Hepatology volume 12, pages 561–571 (2015)Cite this article

Subjects

Key Points

  • Coeliac disease is often accompanied by extraintestinal manifestations, which can be the result of aberrant immune responses but also malabsorption

  • These concurrent conditions can affect various systems and organs, and include manifestations in the skin, musculoskeletal and central nervous system

  • Anaemia, osteoporosis, dermatitis herpetiformis and gluten ataxia are among the most commonly seen characteristics

  • In the paediatric population, coeliac disease can lead to severe growth disorders, such as short stature and delayed puberty due to hypogonadism

Abstract

Coeliac disease is a common disorder that can arise at any age and typically presents with a broad spectrum of symptoms. The disease is thought to be underdiagnosed, in part owing to the fact that coeliac disease is often characterized by associated conditions and extraintestinal manifestations that can misdirect and impede diagnosis. Some of these manifestations are direct consequences of autoimmunity, such as dermatitis herpetiformis or gluten ataxia, whereas others are indirectly related to inflammation and/or malabsorption including anaemia, osteoporosis, short stature and delayed puberty. Any organ from the central nervous system to joints, liver or teeth can be affected. In some cases, extraintestinal symptoms are the only clinical manifestations of coeliac disease or occur in conjunction with diarrhoea and malabsorptive symptoms. An increased awareness among medical practitioners of the variety of extraintestinal manifestations of coeliac disease is essential to improve diagnosis and treatment.

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Figure 1: Extraintestinal manifestations of coeliac disease.
Figure 2: Osteoporosis as a manifestation of coeliac disease.
Figure 3: Dermatitis herpetiformis.
Figure 4: Oral defects as a manifestation of coeliac disease.

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Authors and Affiliations

  1. The Celiac Centre at Beth Israel Deaconess Medical Centre, 330 Brookline Avenue, Boston, 02215, MA, USA

    Daniel A. Leffler

  2. Celiac Disease Centre at Columbia University, 180 Fort Washington Avenue, HP 934, New York, 10032, NY, USA

    Peter H. R. Green

  3. Centre for Celiac Research, Massachusetts General Hospital, 55 Fruit Street, Boston, 02114, MA, USA

    Alessio Fasano

Authors
  1. Daniel A. Leffler
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  2. Peter H. R. Green
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  3. Alessio Fasano
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The authors contributed equally to all aspects in the production of this article.

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Correspondence to Alessio Fasano.

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Competing interests

D.A.L. has received funding from Alba Therapeutics, Alvine Pharmaceuticals, Coronado Bioscience, Inova diagnostics, Ironwood Pharmaceuticals, Sidney Frank Foundation and Shire Pharmaceuticals. P.H.R.G. has received funding from Alba Therapeutics, Alvine Pharmaceuticals and Immusan. A.F. has received funding from Alba Therapeutics, Inova diagnostics, Mead Johnson Nutrition and Pfizer Pharmaceutical.

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Leffler, D., Green, P. & Fasano, A. Extraintestinal manifestations of coeliac disease. Nat Rev Gastroenterol Hepatol 12, 561–571 (2015). https://doi.org/10.1038/nrgastro.2015.131

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