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Case Reports
. 2014 Feb 13:14:29.
doi: 10.1186/1471-230X-14-29.

Ulcerative jejunitis in a child with celiac disease

Terry Sigman  1 , Van-Hung Nguyen, Florin Costea, Ana Sant'Anna, Ernest G Seidman
Affiliations
Case Reports

Ulcerative jejunitis in a child with celiac disease

Terry Sigman et al. BMC Gastroenterol. .

Abstract

Background: Celiac disease can present in children and adults with a variety of manifestations including a rare complication known as ulcerative jejunitis. The latter has been associated with refractory celiac disease in adult onset patients. The objective of this case report is to describe the first pediatric case of ulcerative jejunitis in celiac disease, diagnosed by capsule endoscopy, which was not associated with refractory celiac disease.

Case presentation: The 9 year old girl presented with a history of abdominal pain and vomiting. Laboratory investigations revealed a slightly elevated IgA tissue transglutaminase antibody level in the setting of serum IgA deficiency. Initial upper endoscopy with biopsies was not conclusive for celiac disease. Further investigations included positive IgA anti-endomysium antibody, and positive HLA DQ2 typing. Video capsule endoscopy showed delayed appearance of villi until the proximal to mid jejunum and jejunal mucosal ulcerations. Push enteroscopy with biopsies subsequently confirmed the diagnosis of celiac disease and ulcerative jejunitis. Immunohistochemical studies of the intraepithelial lymphocytes and PCR amplification revealed surface expression of CD3 and CD8 and oligoclonal T cell populations. A repeat capsule study and upper endoscopy, 1 year and 4 years following a strict gluten free diet showed endoscopic and histological normalization of the small bowel.

Conclusion: Ulcerative jejunitis in association with celiac disease has never previously been described in children. Capsule endoscopy was essential to both the diagnosis of celiac disease and its associated ulcerative jejunitis. The repeat capsule endoscopy findings, one year following institution of a gluten free diet, also suggest that ulcerative jejunitis is not always associated with refractory celiac disease and does not necessarily dictate a poor outcome.

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Figures

Figure 1
Figure 1
WIRELESS CAPSULE ENDOSCOPY Initial Exam (Normal Diet). Findings highly suggestive of celiac disease complicated by ulcerative jejunitis. Push enteroscopy suggested to obtain diagnosis and to rule out EATL.
Figure 2
Figure 2
Histological findings on a normal diet. Histological confirmation of celiac disease by push enteroscopy.
Figure 3
Figure 3
Immunohistochemical phenotyping of IELs (at 95 cm in jejunum) at time of diagnosis. Intraepithelial lymphocytosis (IEL), 89 lymphocytes/100 enterocytes with double positivity for CD3 (LEFT) and CD8 (RIGHT), and negative for CD4; consistent with Marsh III A classification.
Figure 4
Figure 4
Histology and immunohistochemical phenotyping of IELs (at 150 cm in jejunum) 6 months after starting GFD. Intraepithelial lymphocytosis (IEL), 40 lymphocytes/100 enterocytes with double positivity for CD3 (LEFT) and CD8 (RIGHT), and negative for CD4; consistent with Marsh I classification.
See this image and copyright information in PMC

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