Ulcerative Jejunitis in Celiac Disease: A Thirty-Year U.S. Experience.

Introduction: Ulcerative jejunitis (UJ) or ulcerative enteritis (UE) is a rare complication of celiac disease (CeD). Guidelines regarding diagnosis and management are missing and these cases have seldom been reported in the United States.

Design: Single center case-series of CeD in which UE developed at a large academic center in the USA. Clinical presentation, diagnosis, treatment, and evolution of disease were collected.

Results: Eight cases were identified (6M/2F, mean age 59.5 (38-77) years). Presentations included intestinal obstruction (n=3), GI hemorrhage (n=3), and malabsorption (n=2). Ulcers were present in the duodenum in 4 patients, and exclusively past the angle of Treitz in only 4 cases, which makes the term ulcerative enteritis (UE) more appropriate than UJ. Six out of eight had T-cell receptor (TCR) clonal gene rearrangements and two had definite aberrant T cells. Corticosteroids were tried in all patients without improvement and 5 underwent surgical resection. Three patients received cladribine. One patient received an autologous stem cell transplant, followed by ruxolitinib. Two were subsequently diagnosed with enteropathy-associated T-cell lymphoma (EATL), including one with cerebral EATL, and 1 died from hemophagocytic syndrome. Two are still alive, including one only on GFD and two were lost to follow-up after surviving at least 30 months post treatment.

Conclusion: UE seems a more appropriate term to describe an ulcerative complication of CeD at high risk of obstruction or bleeding. Steroids were not effective. Treatment outcomes were variable, but with a 50% death rate.