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Immunohistochemical differentiation between chronic enteropathy associated with SLCO2A1 gene and other inflammatory bowel diseases.

ABSTRACT: Background/Aims:We recently identified recessive mutations in the solute carrier organic anion transporter family member 2A1 gene (SLCO2A1) as causative variants of chronic enteropathy associated with SLCO2A1 (CEAS). The aim of this study was to evaluate SLCO2A1 protein expression in the intestinal tissues of patients with CEAS, intestinal Behçet's disease (BD), simple ulcer (SU), and Crohn's disease (CD). Methods:Immunohistochemical staining using a polyclonal anti-SLCO2A1 antibody was performed on the resected intestinal specimens from 13 cases of CD, 9 cases of intestinal BD/SU, and 3 cases of CEAS. The extent of SLCO2A1 expression was determined by counting positively-staining vascular endothelial cells and scored as 0 (no cells), 1 (1%-30% cells), 2 (31%-60%), or 3 (>60%). The intensity of SLCO2A1 expression was scored either as 0 (negative), 1 (intermediate), or 2 (strong). The extent score and intensity score were summed for the final score of 0, 2, 3, 4, or 5. Results:SLCO2A1 protein expression was observed in 1 of 3 cases of CEAS (33%), all 13 cases of CD (100%), and all 9 cases of BD/SU (100%). The mean final expression scores of CEAS, CD, and BD/SU were 1.6 (range, 0-5), 4.8 (range, 4-5), and 4.3 (range, 4-5), respectively. The final expression score in CEAS was significantly lower than in CD (P=0.03). Conclusions:Immunohistochemical staining of the SLCO2A1 protein is considered useful to distinguish CEAS from other inflammatory bowel diseases.

SUBMITTER: Yamaguchi S

PROVIDER: S-EPMC6077302 | biostudies-literature | 2018 Jul

REPOSITORIES: biostudies-literature

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Immunohistochemical differentiation between chronic enteropathy associated with <i>SLCO2A1</i> gene and other inflammatory bowel diseases.

Yamaguchi Satoko S Yanai Shunichi S Nakamura Shotaro S Kawasaki Keisuke K Eizuka Makoto M Uesugi Noriyuki N Sugai Tamotsu T Umeno Junji J Esaki Motohiro M Matsumoto Takayuki T

Intestinal research 20180727 3

<h4>Background/aims</h4>We recently identified recessive mutations in the solute carrier organic anion transporter family member 2A1 gene (<i>SLCO2A1</i>) as causative variants of chronic enteropathy associated with <i>SLCO2A1</i> (CEAS). The aim of this study was to evaluate SLCO2A1 protein expression in the intestinal tissues of patients with CEAS, intestinal Behçet's disease (BD), simple ulcer (SU), and Crohn's disease (CD).<h4>Methods</h4>Immunohistochemical staining using a polyclonal anti- ...[more]

PMID: 30090038

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Project description:ObjectiveChronic enteropathy associated with SLCO2A1 gene (CEAS) is a recently recognized disease. We aimed to evaluate the enterographic findings of CEAS.Materials and methodsAltogether, 14 patients with CEAS were confirmed based on known SLCO2A1 mutations. They were registered in a multicenter Korean registry between July 2018 and July 2021. Nine of the patients (37.2 ± 13 years; all female) who underwent surgery-naïve-state computed tomography enterography (CTE) or magnetic resonance enterography (MRE) were identified. Two experienced radiologists reviewed 25 and 2 sets of CTE and MRE examinations, respectively, regarding the small bowel findings.ResultsIn initial evaluation, eight patients showed a total of 37 areas with mural abnormalities in the ileum on CTE, including 1-4 segments in six and > 10 segments in two patients. One patient showed unremarkable CTE. The involved segments were 10-85 mm (median, 20 mm) in length, 3-14 mm (median, 7 mm) in mural thickness, circumferential in 86.5% (32/37), and showed stratified enhancement in the enteric and portal phases in 91.9% (34/37) and 81.8% (9/11), respectively. Perienteric infiltration and prominent vasa recta were noted in 2.7% (1/37) and 13.5% (5/37), respectively. Bowel strictures were identified in six patients (66.7%), with a maximum upstream diameter of 31-48 mm. Two patients underwent surgery for strictures immediately after the initial enterography. Follow-up CTE and MRE in the remaining patients showed minimal-to-mild changes in the extent and thickness of the mural involvement for 17-138 months (median, 47.5 months) after initial enterography. Two patients required surgery for bowel stricture at 19 and 38 months of follow-up, respectively.ConclusionCEAS of the small bowel typically manifested on enterography in varying numbers and lengths of abnormal ileal segments that showed circumferential mural thickening with layered enhancement without perienteric abnormalities. The lesions caused bowel strictures that required surgery in some patients.

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Immunohistochemical differentiation between chronic enteropathy associated with SLCO2A1 gene and other inflammatory bowel diseases.

Publications

Immunohistochemical differentiation between chronic enteropathy associated with <i>SLCO2A1</i> gene and other inflammatory bowel diseases.

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