Ulcerative Jejunitis in Celiac Disease: A 30-Year US Experience

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.

Case series of CeD in which UE developed at a large academic center in the United States. Clinical presentation, diagnosis, treatment, and evolution of disease were collected.

Eight cases were identified (6 male/2 female, 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 UE more appropriate than UJ. Six of 8 had T-cell receptor clonal gene rearrangements, and 2 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, including 1 with cerebral enteropathy-associated T-cell lymphoma, and 1 died from hemophagocytic syndrome. Two are still alive, including only 1 on GFD and 2 were lost to follow-up after surviving at least 30-month posttreatment.

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.