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Spontaneous Jejunal perforation in coeliac disease: diagnostic dilemma and navigating treatment beyond gluten-free diet in the absence of refractory disease
Abstract
Coeliac disease (CD) is relatively common in the West, affecting up to 1% undergoing serological screening and 0.6% histologically confirmed diagnosis of the population [1]. The pathophysiology involves a complex interplay between genetic susceptibility, environmental, and immunologic factors, resulting in chronic inflammation. 95% of people with CD exhibits the HLA-DQ2 and HLA-DQ8 genes which are crucial for presenting gluten-derived peptides to immune cells. The end result is activation of CD4+ T-helper cells in the lamina propria. These cells release inflammatory cytokines such as interferon-gamma that drive chronic inflammation and ultimately villous atrophy [2].
While treatment is mainly conservative and mandatory avoidance of gluten-containing diet, a small proportion fails to response to this measure, leading to progressive disease and refractory malabsorption. This is called the refractory CD (RCD). Diagnosis of this requires absolute proof that Gluten-Free Diet (GFD) has been adopted for at least 12 months, apart from exclusion of other potential causes that may mimic CD [3].
RCD is categorised into type 1 and type 2, based on the presence of aberrant intraepithelial lymphocytes, with the latter carrying a worse prognosis. Most cases of CD associated Ulcerative Jejunitis (UJ), a chronic immune-mediated deep ulceration within the jejunum is found coexisting with type 2 RCD. Very few presents with UJ alone with no RCD association.
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Date
2025-03
Type
Article
Subject
Gastroenterology, General Surgery
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Muhammad Hafiz Kamarul Bahrin, Hidayatul Nabila Rosaidi, Ammar Mohd Amin, Mirza Faisal Anwar Baig, Martyn Dibb, Spontaneous Jejunal perforation in coeliac disease: diagnostic dilemma and navigating treatment beyond gluten-free diet in the absence of refractory disease, Oxford Medical Case Reports, Volume 2025, Issue 3, March 2025, omae210
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Oxford Medical Case Reports