Classification of intestinal lymphangiectasia with protein-losing enteropathy: White villi type and non-white villi type

Naoki Omiya, Masanao Nakamura, Takeshi Yamamura, Koji Yamada, Asuka Nagura, Toru Yoshimura, Yoshiki Hirooka, Ichiro Hirata, Hidemi Goto

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Abstract

Background/Aims: We classified intestinal lymphangiectasia (IL) into two categories, the white and non-white villi types, and evaluated their clinical characteristics and therapeutic responses. Methods: Of the 988 patients who underwent double-balloon enteroscopy, 14 consecutive patients (7 men and 7 women, median age at onset 34 years) were enrolled with immunohistochemically confirmed IL with protein-losing enteropathy. Results: Enteroscopically the white villi type (n = 8) showed white plaques and white-tipped villi were scattered in the small bowel, while non-white villi type (n = 6) showed that apparently normal but under more detailed observation, low and round villi with a normal color were diffused. The serum albumin levels and fecal α1-antitrypsin clearance before treatment were significantly worse in the non-white villi type (p = 0.017 and 0.039, respectively), whereas the serum immunoglobulin A and M levels were significantly lower in the white villi type (p = 0.010 and 0.046, respectively). At gastroscopy, a non-cirrhotic snakeskin appearance was significantly observed in the non-white villi type (p = 0.015). The corticosteroid response was better in the non-white villi type (p = 0.015). Conclusion: Two distinct subgroups were found in IL. This classification was useful in pathophysiological clustering and in predicting the therapeutic response.

Original languageEnglish
Pages (from-to)155-166
Number of pages12
JournalDigestion
Volume90
Issue number3
DOIs
Publication statusPublished - 25-12-2014

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All Science Journal Classification (ASJC) codes

  • Gastroenterology

Cite this

Omiya, N., Nakamura, M., Yamamura, T., Yamada, K., Nagura, A., Yoshimura, T., Hirooka, Y., Hirata, I., & Goto, H. (2014). Classification of intestinal lymphangiectasia with protein-losing enteropathy: White villi type and non-white villi type. Digestion, 90(3), 155-166. https://doi.org/10.1159/000365987