Impact of Standardized Duodenal Biopsy Sampling on the Diagnosis of Ultrashort Celiac Disease

Ultrashort celiac disease (UCD) is a subtype of celiac disease characterized by villous atrophy confined to the duodenal bulb. Its diagnosis remains challenging due to the histologic particularities of the bulb and the low adherence to recommended biopsy protocols. In a series of 728 adult patients, the prevalence of UCD was 4.5%. Patients presented digestive symptoms comparable to those of conventional celiac disease (CCD), but showed a lower frequency of extraintestinal manifestations, lower levels of IgA anti-tissue transglutaminase antibodies, and a lower prevalence of the HLA-DQ2.5 haplotype, findings consistent with previously published data. Standardization of duodenal bulb biopsy sampling from 2015 onward increased the proportion of endoscopies including bulb biopsies from 36.2% to 90.1%, resulting in a more than sevenfold increase in UCD diagnosis (0.7% vs 5.4%). These results support UCD as a distinct subtype and underscore the importance of standardizing endoscopic procedures and professional training to avoid underdiagnosis.