A Case of Pleuroparenchymal Fibroelastosis with Coexisting Features of Usual Interstitial Pneumonia

肋膜肺實質彈性纖維增生合併尋常性間質性肺炎之影像學／病理學變化的個案：案例報告及文獻回顧

李奕嫻(I-Hsien Lee) ； 謝明書(Min-Shu Hsieh) ； 郭炳宏(Ping-Hung Kuo)

《胸腔醫學》 34卷5期 (2019/10) Pp. 221-229

摘要

Pleuroparenchymal fibroelastosis (PPFE) is a rare idiopathic interstitial pneumonitis. The diagnosis of PPFE can be based on clinical and radiological features, and pathological findings. PPFE can coexist with usual interstitial pneumonia (UIP) on both high-resolution computed tomography (HRCT) and lung biopsy. Therefore, it may cause a diagnostic dilemma in the differentiation of PPFE from idiopathic pulmonary fibrosis (IPF) with upper lung involvement. We report a 71-year-old woman suffering from progressive dyspnea and dry cough for 1 year. HRCT revealed dense pleural thickening and subpleural fibrosis and consolidations in the upper lobes. There was also diffuse subpleural reticulation and traction bronchiectasis/bronchiolectasis. She underwent surgical lung biopsy and the pathology was consistent with PPFE after elastin staining. Histological features of UIP were also found in the upper lobes. Her cough and exertional dyspnea partially improved after off-label treatment with pirfenidone. Our experience with this case suggests that PPFE patients may have HRCT and pathological features of UIP. We also reviewed the differential diagnoses of PPFE and IPF and their clinical outcomes in the literature.

關鍵字

idiopathic pulmonary fibrosis (IPF) ； pleuroparenchymal fibroelastosis (PPFE) ； usual interstitial pneumonia (UIP)

並列摘要

肋膜肺實質彈性纖維增生是一種罕見的間質性肺病，其診斷需要參考臨床、影像學、病理的發現。在高解析度電腦斷層以及病理切片中，可與尋常性間質性肺炎同時存在，因此在鑑別肋膜肺實質彈性纖維增生以及在波及上肺野的特發性肺纖維化時可能遭遇困難。本個案為一位72歲女性，因漸進性乾咳及活動喘一年來就診，高解析度電腦斷層顯現尋常性間質性肺炎型態以外，也在上肺葉有肋膜增厚以及肋膜下纖維化的影像，經胸腔鏡楔形肺葉切片手術後病理診斷為肋膜肺實質彈性纖維增生但併有特發性肺纖維化的特徵，經多團隊會議討論後認為個案傾向肋膜肺實質彈性纖維增生的疾病，但仍給予pirfenidone藥物治療嘗試，病患症狀獲得改善。除呈現本個案診斷過程外，我們亦進行文獻回顧比較特發性肺纖維化及肋膜肺實質彈性纖維增生的差異。

並列關鍵字

特發性肺纖維化 ； 肋膜肺實質彈性纖維增生 ； 高解析度電腦斷層