Type II Refractory Celiac Disease

After excluding common causes of non-responsive celiac disease (NRCD), clinicians should suspect refractory celiac disease (RCD) and determine if a clonal or abnormal population of T-cells is evident in the duodenal mucosa. The presence of this aberrant T-cell population in the absence of lymphoma is diagnostic of type II RCD. This entity, which likely affects less than 0.5% of celiac patients, is often challenging to diagnose and manage and carries substantial morbidity and mortality, mainly enteropathy-associated T-cell lymphoma (EATL). Limited data exist regarding the optimal management of this condition, including enteric and systemic corticosteroids, purine analogues, JAK inhibitors, and stem cell transplantation.