Refractory Celiac Disease: What the Gastroenterologist Should Know.

Area of Science:

• Gastroenterology
• Immunology
• Oncology

Background:

• Refractory celiac disease (RCD) affects fewer than 1% of celiac disease (CD) patients.
• RCD presents as a severe condition with high mortality risks, including intestinal failure, enteropathy-associated T cell lymphoma (EATL), and infections.
• Familiarity with RCD and non-responsive CD (NRCD) is essential for all gastroenterologists managing CD patients.

Purpose of the Study:

• To review the diagnostic and therapeutic approaches for refractory celiac disease (RCD).
• To provide essential knowledge for healthcare providers managing patients with celiac disease and its refractory forms.

Main Methods:

• Literature review summarizing current diagnostic criteria for RCD.
• Analysis of differential diagnoses for gastrointestinal symptoms and villous atrophy.
• Evaluation of treatment strategies for RCD type I and RCD type II.

Main Results:

• Diagnostic workup and differentiation between RCD types are complex, often needing specialized centers.
• Immunosuppression is effective for RCD type I.
• Current treatments for RCD type II offer limited efficacy, mainly transient improvement and delayed EATL development.

Conclusions:

• All gastroenterologists must understand RCD basics due to its potential severity and complications.
• Accurate diagnosis and appropriate management, though complex, are critical for improving outcomes in refractory celiac disease.
• Further research is needed for effective RCD type II therapies.