Refractory celiac disease was recently subdivided into 2 subtypes, known as refractory celiac disease 1 and 2.
This subdivision is based on a normal or abnormal phenotype of intraepithelial lymphocytes, respectively.
It is not clear, however, if these 2 entities differ in their presentation at diagnosis or long-term outcome.
Dr Georgia Malamut and colleagues from France compared the clinical and biological characteristics of refractory celiac disease 1 and refractory celiac disease 2 at diagnosis.
In addition, the team assessed the risk of developing an overt lymphoma, and the predictive factors of survival.
The team analyzed medical files of 14 patients with refractory celiac disease 1, and 43 with refractory celiac disease 2 retrospectively.
Predictive factors of overt lymphoma and survival were studied in univariate and multivariate analyses.
|The 5-year survival rate was 93% in refractory celiac disease type 1|
The researchers found that at diagnosis, malnutrition, ulcerative jejunitis, and lymphocytic gastritis were more common in patients with refractory celiac disease 2 than refractory celiac disease 1.
Overt lymphomas occurred in 2 patients with refractory celiac disease 1, and 16 with refractory celiac disease 2.
In the univariate analysis, abnormal intraepithelial lymphocytes phenotype and increased age at diagnosis of refractory celiac disease were predictive factors for overt lymphoma.
The team found that abnormal intraepithelial lymphocytes phenotype, clonality, and overt lymphoma predicted short survival time.
Only abnormal intraepithelial lymphocytes phenotype, and overt lymphoma were predictive in the multivariate analysis.
The 5-year survival rate was 93% in patients with refractory celiac disease 1, and 44% with refractory celiac disease 2.
Dr Malamut’s team concluded, “Refractory celiac disease 2 has a much more severe presentation and prognosis than patients with refractory celiac disease 1.”
“Less than 44% of patients with refractory celiac disease 2 survive 5 years after diagnosis.”
“Abnormal intraepithelial lymphocytes phenotype is a predictive factor but not a necessary condition for the development of overt lymphoma.”