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Does cystic fibrosis make susceptible to celiac disease?

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Abstract

Patients with cystic fibrosis (CF) have a higher incidence of celiac disease (CD) than the healthy population; however, the actual incidence of coexisting CF and CD is unclear. In this report, we aimed to evaluate the frequency of CD and CF coexistence and to assess the clinical findings of affected patients during follow-up. We conducted a retrospective review of patients with CF to reveal the frequency of CD and also investigated the clinical characteristics and clinical response to gluten-free diet in patients with CD. The incidence of CD in 515 patients with CF was 1.4%. The median age at the time of CF diagnosis was 2 months (1–6 months). CD was diagnosed in six patients with poor weight gain, fatty stools, and low z score for BMI and one patient with poor weight gain despite a high protein and calorie diet and pancreatic enzyme replacement. The median age of CD diagnosis was 8 years (2–12 years). Except for one patient who was recently diagnosed, the other six patients gained weight and their accompanying symptoms resolved after starting a gluten-free diet.

Conclusion: CD should be investigated in patients with CF in the presence of inadequate weight and/or height gain or poor control of malabsorption symptoms despite appropriate and adequate nutritional and enzyme replacement treatment.

What is Known:

• CFTR dysfunction may be a risk factor for CD, due to increased intestinal permeability and intestinal inflammation, pancreatic exocrine insufficiency that results in higher antigen load and increased antibodies against to nutritional antigens such as anti-gliadin IgA antibodies.

• Although coexistence of CF and CD are rare in the same patient; there is still no consensus on when children with CF should be screened for CD.

What is New:

• Physicians should consider the investigation of CD in patients with CF, in the presence of inadequate weight and/or height gain or poor control of malabsorption symptoms despite appropriate and adequate nutritional and enzyme replacement treatment.

• CFTR dysfunction has been emphasized to develop susceptibility to CD, and patients with CF who have persistent gastrointestinal symptoms despite appropriate and adequate nutritional and enzyme replacement treatment should be screened for CD.

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The data and materials are available.

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Abbreviations

BECN-1:

Beclin-1

BMI:

Body mass index

CD:

Celiac disease

CF:

Cystic fibrosis

CFTR:

Cystic fibrosis transmembrane regulator

EMA-IgA:

Endomysium antibody IgA

ESPGHAN:

European Society for Pediatric Gastroenterology Hepatology and Nutrition

FEV1:

Forced expiratory volume 1

HLA:

Human leukocyte antigen

NBD-1:

Nucleotide-binding domain 1

SD:

Standard deviation

tTG-IgA:

Tissue transglutaminase immunoglobulin Ig A

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Authors and Affiliations

  1. Department of Pediatric Pulmonology, Hacettepe University Faculty of Medicine, Ankara, Turkey

    Nagehan Emiralioglu, Dilber Ademhan Tural, Beste Ozsezen, Birce Sunman, Ebru Yalcin, Deniz Dogru, Uğur Ozcelik & Nural Kiper

  2. Department of Pediatric Gastroenterology, Hacettepe University Faculty of Medicine, Ankara, Turkey

    Hayriye Hizarcioglu Gulsen, Yasin Maruf Ergen & İncinur Saltık Temizel

Authors
  1. Nagehan Emiralioglu
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  2. Dilber Ademhan Tural
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Contributions

NE, DAT, HHG, YME, İST, BÖ, BS, EY, DD, UÖ, and NK have made substantial contributions to the design of the work and the interpretation of data for the work.

NE, HHG, DD, UÖ, NK, EY, and İST have revised the manuscript critically for important intellectual content.

NE, DAT, HHG, BÖ, BS, EY, DD, UÖ, and NK have made contributions for final approval of the version to be published.

NE, DAT, HHG, YME, İST, BÖ, BS, EY, DD, UÖ, and NK had the agreement to be accountable for all aspects of the work in ensuring that questions related to the accuracy or integrity of any part of the work are appropriately investigated and resolved.

Corresponding author

Correspondence to Nagehan Emiralioglu.

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Ethics approval

University local ethics committee approved the study (Number: GO 18/473-31).

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Written informed consent was obtained from all patients and their families.

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Written informed consent was obtained for publication.

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The authors declare no competing interests.

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Communicated by Peter de Winter

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Emiralioglu, N., Ademhan Tural, D., Hizarcioglu Gulsen, H. et al. Does cystic fibrosis make susceptible to celiac disease?. Eur J Pediatr 180, 2807–2813 (2021). https://doi.org/10.1007/s00431-021-04011-4

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