Internal Medicine
Online ISSN : 1349-7235
Print ISSN : 0918-2918
ISSN-L : 0918-2918
CASE REPORTS
Protein-losing Enteropathy Caused by Intestinal or Colonic Lymphangiectasia Complicated by Sporadic Lymphangioleiomyomatosis: A Report of Two Cases
Koichi NishinoKaku YoshimiTomoyoshi ShibuyaTakuo HayashiKeiko MitaniEtsuko KobayashiMasako IchikawaTetsuhiko AsaoYohei SuzukiTadashi SatoSatomi ShiotaYuzo KodamaKazuhisa TakahashiKuniaki Seyama
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JOURNAL OPEN ACCESS

2017 Volume 56 Issue 8 Pages 943-948

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Abstract

This report describes two patients with sporadic lymphangioleiomyomatosis complicated by protein-losing enteropathy (PLE). Imaging studies indicated retroperitoneal lymphangioleiomyomas and abnormalities of the adjacent digestive tract. Endoscopic mucosal biopsy revealed colonic lymphangiectasia in one patient; whereas the site in the other patient was intestinal. Treatment with sirolimus led to the complete resolution of PLE within several months; additionally, marked shrinkage was observed in the lymphangioleiomyomas of both cases. These findings suggest that colonic or intestinal lymphatic congestion due to neighboring lymphangioleiomyomas was the mechanism for the development of PLE. At the time of writing this report, the beneficial effect of sirolimus has lasted for more than 3 years.

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© 2017 by The Japanese Society of Internal Medicine
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