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Ulcerative jejunitis in a child with celiac disease.

Sigman T, Nguyen VH, Costea F, Sant'Anna A, Seidman EG - BMC Gastroenterol (2014)

Bottom Line: Initial upper endoscopy with biopsies was not conclusive for celiac disease.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.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Pediatric Gastroenterology, Montreal Children's Hospital, Faculty of Medicine, McGill University, 2300 Tupper St, Montreal, Quebec H3H 1P3, Canada. terry.sigman@muhc.mcgill.ca.

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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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.
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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.

Mentions: Institution of a strict GFD for 6 months led to clinical improvement, with considerably less pain and cessation of vomiting. Moreover, her serum tTG normalized (<3 U/ml). However, a repeat CE again revealed delayed appearance of villi until the mid jejunum and mucosal ulcerations in the proximal jejunum without active bleeding, consistent with persistent ulcerative jejunitis. Biopsies obtained by repeat push enteroscopy after 6 months on a GFD showed normal villous architecture in the duodenum and jejunum down to 150 cm, with an IEL count of 12/100 enterocytes per HPF in the duodenum (Marsh 0) and 40-46/100 per HPF in the jejunum at 130 cm and 150 cm respectively (Marsh I). The biopsies were subsequently sent for lymphocytic identification and PCR amplification studies for T cell rearrangement on fresh tissue. PCR amplification studies were also done on paraffin embedded tissue from the samples at diagnosis. On the sample from initial diagnosis prior to GFD, a T-cell clonal population was observed although it stained positive for surface expression of both CD3 and CD8 (Figure 3). The specimen obtained after 6 months on a GFD showed oligoclonal T-cell populations and still stained positive for surface expression of both CD3 and CD8 (Figure 4). Since there was definite improvement histologically, a decision was made to continue the strict GFD and to re-evaluate by CE and histology after another 6 months.


Ulcerative jejunitis in a child with celiac disease.

Sigman T, Nguyen VH, Costea F, Sant'Anna A, Seidman EG - BMC Gastroenterol (2014)

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.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4016221&req=5

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.
Mentions: Institution of a strict GFD for 6 months led to clinical improvement, with considerably less pain and cessation of vomiting. Moreover, her serum tTG normalized (<3 U/ml). However, a repeat CE again revealed delayed appearance of villi until the mid jejunum and mucosal ulcerations in the proximal jejunum without active bleeding, consistent with persistent ulcerative jejunitis. Biopsies obtained by repeat push enteroscopy after 6 months on a GFD showed normal villous architecture in the duodenum and jejunum down to 150 cm, with an IEL count of 12/100 enterocytes per HPF in the duodenum (Marsh 0) and 40-46/100 per HPF in the jejunum at 130 cm and 150 cm respectively (Marsh I). The biopsies were subsequently sent for lymphocytic identification and PCR amplification studies for T cell rearrangement on fresh tissue. PCR amplification studies were also done on paraffin embedded tissue from the samples at diagnosis. On the sample from initial diagnosis prior to GFD, a T-cell clonal population was observed although it stained positive for surface expression of both CD3 and CD8 (Figure 3). The specimen obtained after 6 months on a GFD showed oligoclonal T-cell populations and still stained positive for surface expression of both CD3 and CD8 (Figure 4). Since there was definite improvement histologically, a decision was made to continue the strict GFD and to re-evaluate by CE and histology after another 6 months.

Bottom Line: Initial upper endoscopy with biopsies was not conclusive for celiac disease.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.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.

View Article: PubMed Central - HTML - PubMed

Affiliation: Division of Pediatric Gastroenterology, Montreal Children's Hospital, Faculty of Medicine, McGill University, 2300 Tupper St, Montreal, Quebec H3H 1P3, Canada. terry.sigman@muhc.mcgill.ca.

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.

Show MeSH
Related in: MedlinePlus