Diagnosis and management of refractory celiac disease: a systematic review.
BACKGROUND
Refractory celiac disease is defined by persisting malabsorptive symptoms in spite of a strict gluten free diet for at least 6 to 12 months. Alternatives to gluten free diet seem to be still controversial.


OBJECTIVE
To describe the clinical and epidemiologic aspects of refractory celiac disease, and to identify therapeutic options in this condition.


METHODS
Systematic review and critical analysis of observational studies, clinical trials and case reports that focused on diagnosis and management of refractory celiac disease.


RESULTS
Refractory celiac disease can be classified as type 1 or type 2 according to the phenotype of intraepithelial lymphocytes. Great complications such as enteropathy-associated T-cell lymphoma may occur in a subgroup of these patients mainly in refractory celiac disease type 2. Curative therapies are still lacking.


CONCLUSIONS
Refractory celiac disease remains a diagnosis of exclusion. Its prognosis remains still dismal by the absence yet of curative therapies. However, some new treatments seem to hold promise during few cohort-studies.
Asma
A
Labidi
Meriem
M
Serghini
Sami
S
Karoui
Jalel
J
Boubaker
Azza
A
Filali
D002446:Celiac Disease / Q000175:diagnosis* / Q000453:epidemiology / Q000628:therapy*; D003937:Diagnosis, Differential; D055050:Diet, Gluten-Free; D006801:Humans; D011379:Prognosis; D017211:Treatment Failure