A subset of patients with coeliac disease continues to experience symptoms and intestinal villous damage despite prolonged, strict adherence to a gluten-free diet. When other causes have been excluded and the intraepithelial lymphocyte profile is normal, this presentation defines a specific clinical sub-type requiring a targeted management approach.
Refractory coeliac disease type 1 (RCD-I) is defined by the persistence or recurrence of symptoms and villous atrophy after at least 12 months on a strict gluten-free diet, in the absence of other causes, with a normal (polyclonal or oligoclonal) intraepithelial lymphocyte immunophenotype. This distinguishes RCD-I from other refractory presentations and informs the choice of therapy.
Retrospective and longitudinal evidence supports open-capsule budesonide as first-line pharmacological therapy for RCD-I, with nutritional support added when clinically needed. The complete regimen — including sequencing and the full clinical algorithm — is available in the structured protocol below.
DOI: 10.1002/ueg2.70195
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