Successful novel use of tofacitinib for type II refractory coeliac disease

BMJ Case Rep. 2022 Apr 13;15(4):e244692. doi: 10.1136/bcr-2021-244692.

Abstract

Refractory coeliac disease (RCD) occurs when patients with confirmed CD have continuous or recurrent malabsorption and enteropathy after at least 12 months on a gluten-free diet. Differentiating between type I and type II RCD is key as the latter is associated with T-cell aberrancy and considered prelymphoma, with high mortality rates. Current treatment regimens for type II RCD include corticosteroids, biologics and chemotherapy, but there are no proven therapies for this serious condition. Our patient is a middle-aged woman who developed postpartum type II RCD. When she failed multiple drug classes, we did a trial of tofacitinib. Our clinical experience with use of a janus kinase inhibitor was successful, with no associated adverse events. This is the first report in the literature of RCD remission in response to tofacitinib. The use of this novel agent shows promise in reversing this potentially fatal condition.

Keywords: Coeliac disease; Drugs: gastrointestinal system; Malabsorption; Nutrition; Small intestine.

Publication types

  • Case Reports

MeSH terms

  • Celiac Disease* / diagnosis
  • Diagnosis, Differential
  • Diet, Gluten-Free
  • Female
  • Humans
  • Middle Aged
  • Piperidines
  • Pyrimidines
  • T-Lymphocytes / pathology

Substances

  • Piperidines
  • Pyrimidines
  • tofacitinib