53 / F,；cough and dyspnea for 5 years

53歲，女性,；咳嗽及呼吸困難5年,。

圖1 胸片正位

圖2 胸片側(cè)位

圖3 胸部CT肺窗橫斷面

圖4 胸部CT肺窗橫斷面

圖5 胸部CT肺窗橫斷面

圖6 胸部CT肺窗橫斷面

圖7 胸部CT肺窗冠狀面

圖8 胸部CT肺窗冠狀面

圖9 胸部CT肺窗冠狀面

Figs 1-2. Chest PA and lateral view show increased reticular opacities in both lungs and marked apical pleural thickening.
圖1-2.胸片正側(cè)位顯示兩肺高密度網(wǎng)格影，肺尖胸膜增厚顯著,。

Fig 3-6. Lung window setting of the chest CT scan shows pleural and subpleural thickening with severe fibrotic changes in the marginal parenchyma withapicocaudal distribution.
圖3-6.胸部CT肺窗顯示兩肺邊緣胸膜及胸膜下增厚,，伴重度纖維化，肺尖分布為主,。

Figs 7-9. Coronal reformatted images show marked apical pleural thickening,volume loss of the upper lungs, and peripheral consolidation.
圖7-9.冠狀面重建圖像顯示肺尖胸膜顯著增厚,，肺尖容積減小，外周實(shí)變,。

Note the evolution between 2014 and 2016 (at the time of diagnosis).

注意2014至2016年間疾病進(jìn)展過程,。

She underwent VATS biopsy, and pathologic findings revealed subpleural dense fibrosis with elastosis and patchy lymphocytic infiltration, suggesting pleuroparenchymal fibroelastosis (PPFE).
患者行胸腔鏡活檢，病理表現(xiàn)證實(shí)胸膜下致密纖維化伴彈力纖維增生和片狀淋巴細(xì)胞浸潤,，提示胸膜肺實(shí)質(zhì)彈力纖維癥,。

Her sister was also diagnosed with the same disease.

姐姐被診斷為患同樣疾病。

PPFE is a rare entity that has been recently included in the official American Thoracic Society/European Respiratory Society (ATS/ERS) statement in 2013 as a group of rare idiopathic interstitial pneumonias (IIPs).
PPFE是一種罕見病,，在2013年由美國胸科學(xué)會(huì)（ATS）/歐洲呼吸病學(xué)會(huì)（ERS）將其歸為罕見特發(fā)性間質(zhì)性肺炎,。

PPFE is characterized by pleural and subpleural parenchymal thickening due to elastic fiber proliferation, mainly in the upper lobes.
PPFE是由于彈力纖維增生，以胸膜及胸膜下肺實(shí)質(zhì)增厚為特征,，上肺葉為主,。

Pathologically, interstitial fibrosis takes the form of a dense consolidation with some preservation of alveolar septal outlines and demonstrates a distinctly abrupt interface withresidual normal lung.
病理上，間質(zhì)纖維化表現(xiàn)為致密實(shí)變伴部分肺泡間隔輪廓?dú)埩?，并與殘余正常肺界面光整,。

Unrecognized cases of PPFE may be incorrectly diagnosed as sarcoidosis, atypical idiopathic pulmonary fibrosis, or other unclassifiable interstitialpneumonias.
未識(shí)別的PPFE病例可能被誤診為結(jié)節(jié)病,、不典型特發(fā)性肺纖維化或其他未分類間質(zhì)性肺炎。

The etiology of the disease is unclear, although some cases have been associated as a complication after bone marrow transplantation, lung transplantation, chemotherapy, and recurrent respiratory infections.
盡管在骨髓移植,、肺移植,、化療和反復(fù)呼吸道感染后，PPFE可作為并發(fā)癥出現(xiàn),，但其病因尚不清楚,。

The patients usually report progressive dyspnea and dry cough; Better awareness of the disease with its clinical and radiologic features can help with optimal management by the multidisciplinary team.
患者常表現(xiàn)為進(jìn)行性呼吸困難和干咳；更好地了解該病的臨床和放射學(xué)特征可以幫助MDT優(yōu)化管理,。

Clinical outcome in PPFE is variable with a significant number of patients demonstrating progressive decline and death.
PPFE的臨床結(jié)果不一,，大量患者表現(xiàn)出進(jìn)行性衰退和死亡。

Survival characteristics depend on the stage of the disease at presentation. Aside from lung transplantation, there is no demonstrated effective treatment.
生存特征取決于癥狀明顯期,。除肺移植外,，尚無有效的治療方法。

Radiological differential diagnoses may include chronic hypersensitivity pneumonitis, atypical usual interstitial pneumonitis, collagen vascular diseases, irradiation injury, asbestos exposure, prominent apical cap fibrosis, and sarcoidosis.
放射學(xué)鑒別診斷包括慢性過敏性肺炎,、不典型常見間質(zhì)性肺炎,、膠原血管疾病、放射性損傷,、石棉暴露,、肺尖冒纖維化和結(jié)節(jié)病。

感謝Hae Jin Kim, MD. Tae Jung Kim, MD. / Samsung Medical center