Koguchi Nobuo, Okada Asuka, Choh Sumito, Katayama Kumiko, Takenaka Hideaki, Tomoda Koichi, Kimura Hiroshi
Department of Respiratory Medicine, Saiseikai Suita Hospital, Japan.
Intern Med. 2016;55(10):1331-5. doi: 10.2169/internalmedicine.55.5903. Epub 2016 May 15.
A 75-year-old woman was referred to our hospital with the chief symptom of dyspnea. Chest computed tomography revealed lymphadenopathy, emphysema, and honeycombing. Sarcoidosis was diagnosed due to an elevated serum ACE level and the findings of a lymph-node biopsy. Her smoking history, radiography findings, and impaired gas exchange indicated combined pulmonary fibrosis and emphysema (CPFE). Raynaud's phenomenon gradually appeared, and we also diagnosed her with systemic sclerosis (SSc). Right heart catheterization revealed pulmonary hypertension (PH). Smoking was assumed to be the chief cause, but SSc may also induce the development of CPFE. Severe PH induced by CPFE or SSc was present, but the influence of sarcoidosis also could not be ignored.
一名75岁女性因呼吸困难的主要症状被转诊至我院。胸部计算机断层扫描显示有淋巴结病、肺气肿和蜂窝状改变。由于血清血管紧张素转换酶（ACE）水平升高以及淋巴结活检结果，诊断为结节病。她的吸烟史、影像学检查结果和气体交换受损提示合并存在肺纤维化和肺气肿（CPFE）。雷诺现象逐渐出现，我们还诊断她患有系统性硬化症（SSc）。右心导管检查显示有肺动脉高压（PH）。推测吸烟是主要原因，但SSc也可能促使CPFE的发展。存在由CPFE或SSc引起的严重PH，但结节病的影响也不容忽视。
