Refractory Celiac Disease; Diagnosis, Treatment and Clinical Manifestations

Celiac disease as an autoimmune disease is predisposes in genetically susceptible subjects due to the consumption of wheat and other grains containing gluten and as a result of immunological responses, villous atrophy, mucosal hyperplasia, and lymphocytic infiltration will be occurred. The only treatment is a lifelong gluten-free diet.
Most patients with celiac disease respond to gluten-free diet (GFD). But in a small percentage of patients, despite full compliance with gluten-free diet, symptoms and mucosal atrophy are remains. When other causes of mucosal atrophy are rejected, the diagnosis of refractory celiac disease (RCD) is suggested. Based on the abnormality in population of intraepithelial lymphocytes (IEL), RCD is divided into two types 1; (RCD I) and type 2 (RCDII). Prognosis, clinical symptoms and endoscopic findings of RCD I are better and milder than the RCD II. Treatment of RCD I is based on immunosuppressive therapy and RCD II is mostly based on nutritional support and chemotherapeutic agents. In this review the clinical characteristics, diagnostic and treatment approach of RCD will be reviewed.