Refractory Celiac Disease

Abstract

Purpose of review: To review the epidemiology, pathophysiology, diagnosis, management, and prognosis of refractory celiac disease, with a specific emphasis on recent literature.

Recent findings: While the pathophysiology of type I refractory celiac disease remains unclear, there have been advances in the understanding of the pathophysiology of type II refractory celiac disease. This has included recognition of the significant role of interleukin-15 and somatic mutations in JAK1 or STAT3 in the proliferation of aberrant T cells. This in turn has led to potential novel therapies targeting these factors, one of which has reached the clinical trial stage. The morbidity and mortality associated with type II refractory celiac disease remain significant; however, recent advances in the understanding of the pathophysiology of this condition have led to potential therapeutic options that should be investigated.

Keywords: Budesonide; Enteropathy-associated T cell lymphoma; Flow cytometry; IL-15; Ulcerative jejunitis.