Type I Refractory Celiac Disease

Type I refractory celiac disease (RCD) affects about 1% of patients with celiac disease (CeD) and is characterized by ongoing signs and symptoms of malabsorption along with enteropathy in patients with confirmed CeD consuming a strict gluten-free diet (GFD), in the absence of other causes of villous atrophy or malignant complications. It is differentiated from type II RCD by the absence of abnormal immunophenotyping of intraepithelial lymphocytes (IEL). Management, which is based on case series and expert opinion only, consists of systemic corticosteroid therapy (prednisone or budesonide) and steroid-sparing agents, and prognosis when compared with type II RCD is favorable.