修格連氏症候群合併肺淋巴管肌瘤增生：是同時罹患兩種獨立疾病，或同為免疫相關的疾病？病例報告

Sjögren's Syndrome in a Patient with Lymphangioleiomyomatosis: Two Independently Co-existing or Immunologically Related Diseases?

在高解析度電腦斷層掃描照相（HRCT）有多發性薄壁囊腫，均勻地分佈於肺部中，是肺淋巴管肌瘤（lymphangioleiomyomatosis. LAM）的典型發現。肺淋巴管肌瘤並未有報告過與任何自體免疫疾病有相關。在這份病例報告中，一位43歲臺灣婦女同時患有肺淋巴管肌瘤和修格連氏症候群，而修格連氏症候群在肺部的侵犯很少以多發性薄壁囊腫來表現，且肺淋巴管肌瘤在侵犯的器官表現有transforming growth factor beta 1 （TGFβ1），和自體免疫疾病如修格連氏症候群，可能有相關存在。因此，這份病例報告有助於瞭解在肺淋巴管肌瘤的致病機轉中，自體免疫活化所扮演的角色。

Multiple thin-walled cysts evenly distributed throughout the lung fields in high-resolution computed tomography (HRCT) are characteristic findings of Jymphangioleiomyomatosis (LAM). LAM has not been related to any autoimmune disorder. In this report, we presented a 43 year-old Chinese woman who developed both LAM and Sjögren's syndrome (SS), a disease that may rarely manifest multiple cystic lesions in its lung involvement. Together with the expression of transforming growth factor beta 1 (TGF-β1) in involved organs, a link between LAM and autoimmune disorders like SS may exist. This report is therefore of help in understanding the role of autoimmune reactivation in the development or pathogenesis of LAM.