一般社団法人日本呼吸器学会 公式サイト
日本呼吸器学会英文誌 Respiratory Investigation
日本呼吸器学会誌 増刊号 学術講演会プログラム 抄録集

Abstract

Full Text of PDF Full Text of PDF (507k)
Article in Japanese

Case Report

A case of lymphangioleiomyomatosis with refractory chylous pleural effusions and ascites that was treated with sirolimus

Hiroyoshi Yamauchi  Masashi Bando  Naoko Yoshizumi  Haruna Kogawara  Hideaki Yamasawa  Yukihiko Sugiyama 

Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University

ABSTRACT

A 50-year-old woman with a chief complaint of abdominal pain presented at another hospital. She showed numerous thin-walled lung cysts and abdominal lymphadenopathy on computed tomography. A biopsy of abdominal lymph nodes was performed at the other hospital, and she was diagnosed as angiomyolipoma. Lastly she was diagnosed as lymphangioleiomyomatosis. After a biopsy of abdominal lymph nodes, she had considerable chylous pleural effusions and ascites accumulation. She was treated with a fat-restricted diet and drainage; however, the chylous pleural effusions and ascites were refractory. After she was transferred to our hospital, we began sirolimus therapy with 1 mg/day. Nine days after beginning sirolimus, the chylous pleural effusions and ascites were reduced immediately. Mild stomatitis, rash, and hyperlipidemia were complications resulting from the sirolimus treatment, but we could control these complications. She has currently received sirolimus treatment for approximately 9 months, and the chylous pleural effusions and ascites have not recurred. In this case, sirolimus treatment resulted in the cessation of frequent drainage of uncontrolled pleural effusion and ascites.

KEYWORDS

Lymphangioleiomyomatosis  Sirolimus  Refractory chylous pleural effusions and ascites 

Received 6 Oct 2015 / Accepted 22 Dec 2015

AJRS, 5(2): 101-105, 2016

Google Scholar