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

in The American journal of gastroenterology by Yevgen Chornenkyy, Masa Peric, David Marin Flores, Yuho Ono, Shweta A Shinagare, Katelyn Dannheim, Sarah Shannahan, Shana Rakowsky, Saja Asakrah, Monika Vyas, Jon Arnason, Daniel Leffler, Ciaran Kelly, Rupa Mukherjee, Amelie Therrien

TLDR

  • The study reports on 8 cases of ulcerative enteritis in patients with celiac disease, highlighting the severity and variability of treatment outcomes.
  • The term 'ulcerative enteritis' may be more appropriate than 'ulcerative jejunitis' due to the location of the ulcers.

Abstract

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.

Overview

  • The study aimed to investigate a rare complication of celiac disease, ulcerative jejunitis (UJ) or ulcerative enteritis (UE), and the lack of guidelines for diagnosis and management.
  • Researchers analyzed a case series of 8 patients with Coeliac disease who developed UE at a large academic center in the United States.
  • The study highlights the importance of accurate diagnosis and effective treatment, as patients with UE are at high risk of intestinal obstruction or bleeding and poor treatment outcomes.

Comparative Analysis & Findings

  • The study found that the clinical presentations included intestinal obstruction, GI hemorrhage, and malabsorption, with ulcers present in the duodenum in 4 patients and exclusively past the angle of Treitz in 4 cases.
  • The majority of patients (6/8) had T-cell receptor clonal gene rearrangements, and 2 had definite aberrant T cells, indicating a potential link between UE and T-cell lymphoma.
  • The treatment outcomes were variable, with corticosteroids being ineffective, and 5 patients undergoing surgical resection. Two patients were diagnosed with enteropathy-associated T-cell lymphoma, and one patient died from hemophagocytic syndrome.

Implications and Future Directions

  • The study highlights the need for standardized guidelines for diagnosis and management of UE, a rare but potentially severe complication of celiac disease.
  • Further research is needed to understand the underlying mechanisms of UE and to develop more effective treatments, potentially involving immunomodulatory therapy and targeted therapies.
  • The study also underscores the importance of close monitoring and follow-up for patients with UE, particularly those with T-cell receptor clonal gene rearrangements, to detect potential lymphoma development early.
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