Zhang Ning, Li Qiaolian, Shao Changzhou
These authors share co-first authorship.
Shanghai Institute of Respiratory Diseases, Department of Pulmonary Medicine, Zhongshan Hospital, Fudan University, Shanghai 200032, China.
Sarcoidosis Vasc Diffuse Lung Dis. 2018;35(4):390-394. doi: 10.36141/svdld.v35i4.7077. Epub 2020 Mar 9.
Pulmonary alveolar proteinosis (PAP) is a rare interstitial lung disease classified into congenital form, autoimmune form and secondary form. Secondary PAP is caused by underlying conditions including solid malignancies. Few cases reported PAP associated with lung cancers. To show the clinical features of PAP with adenocarcinom, tried to seek for possible mechanism to explain whole clinical course. Reported a case of PAP associated with lung adenocarcinom, and also reviewed the relevant literature on PAP. The patient suffered from intermittent cough, fever, shortness of breath, thoracalgia or hemoptysis. Blood gas analysis showed hyoxemia. Spirometric abnormality is mildly restrictive defect. High-resolution computed tomography (HRCT) showed patchy, ground-glass opacities with interlobular septal thickening called as "crazy-paving" pattern. Positron emission tomography/computed tomography (PET/CT) revealed a nodule with characteristics of lobulation and spiculation in the right lung apex section and diffuse consolidation shadow spreading over rest of lung field. Bronchoalveolar lavage fluid (BALF) showed a large amount of amorphous red-dyed materials and a few alveolar macrophages scattered in endoalveolar space with PAS positive. Transbronchial lung biopsy found adenocarcinoma. Wedge resection with mediastinal lymphnode and then 2 cycles of postoperative chemotherapy was carried out. No ground-glass opacities were found in his chest CT pictures in the next nine months. This result may support the theory that lung cancer cells cause quantitative or functional damage to alveolar macrophages, which trend to secondary PAP. The patient had typical clinical features of PAP. PAP may be secondary to lung cancer. .
肺泡蛋白沉积症（PAP）是一种罕见的间质性肺疾病，分为先天性、自身免疫性和继发性三种类型。继发性PAP由包括实体恶性肿瘤在内的基础疾病引起。很少有病例报道PAP与肺癌相关。为了展示合并腺癌的PAP的临床特征，试图寻找解释整个临床过程的可能机制。报告了一例合并肺腺癌的PAP病例，并回顾了PAP的相关文献。该患者有间歇性咳嗽、发热、气短、胸痛或咯血症状。血气分析显示低氧血症。肺功能检查异常为轻度限制性通气功能障碍。高分辨率计算机断层扫描（HRCT）显示斑片状磨玻璃影伴小叶间隔增厚，即所谓的“铺路石征”。正电子发射断层扫描/计算机断层扫描（PET/CT）显示右肺尖段有一个具有分叶和毛刺特征的结节，以及弥漫性实变影蔓延至肺野其余部分。支气管肺泡灌洗（BALF）液显示大量无定形红色染色物质，少量肺泡巨噬细胞散在于肺泡腔内，过碘酸雪夫（PAS）染色阳性。经支气管肺活检发现腺癌。进行了纵隔淋巴结楔形切除术，然后进行了2个周期的术后化疗。在接下来的九个月里，其胸部CT图像中未发现磨玻璃影。这一结果可能支持肺癌细胞对肺泡巨噬细胞造成数量或功能损害从而导致继发性PAP的理论。该患者具有典型的PAP临床特征。PAP可能继发于肺癌。
