Recent advances in celiac disease and refractory celiac disease

F1000Res. 2019 Jun 26:8:F1000 Faculty Rev-969. doi: 10.12688/f1000research.18701.1. eCollection 2019.

Abstract

Celiac disease (CeD), defined as gluten-induced enteropathy, is a frequent and largely underdiagnosed disease. Diagnosis relies on the detection of highly specific serum IgA anti-transglutaminase auto-antibodies and on the demonstration of duodenal villous atrophy. Treatment necessitates a strict gluten-free diet, which resolves symptoms and enables histological recovery. However, regular follow-up is necessary to assess mucosal healing, which emerges as an important prognostic factor. Recent work on CeD pathogenesis has highlighted how the cross-talk between gluten-specific CD4 + T cells and interleukin-15 can activate cytotoxic intraepithelial lymphocytes and trigger epithelial lesions. Moreover, acquisition by a subset of intraepithelial lymphocytes of somatic gain-of-function mutations in the JAK-STAT pathway was shown to be a decisive step in the progression toward lymphomas complicating CeD, thus opening new therapeutic perspectives for these rare but life-threatening complications.

Keywords: celiac disease; refractory celiac disease.

Publication types

  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Celiac Disease* / immunology
  • Celiac Disease* / metabolism
  • Celiac Disease* / therapy
  • Diet, Gluten-Free
  • Disease Progression
  • Glutens
  • Humans
  • Transglutaminases

Substances

  • Glutens
  • Transglutaminases

Grants and funding

The laboratory of Intestinal Immunity is supported by INSERM and by grants from Agence Nationale pour la Recherche (ANR) (IRAC, COELAR), Association pour la Recherche Contre le Cancer (ARC PGA1-RF20180206809), la Fondation Princesse Grace, and the French Association of patients intolerant to gluten (AFDIAG). SC is supported by grants from ANR and AFDIAG. Institut Imagine is supported by Investissement d’Avenir ANR-10-IAHU-01.