Makovická Mária, Vrbenská Adela, Javorská Brigita, Durcová Barbora, Makovický Peter, Škarda Jozef, Muri Jozef
Cesk Patol. 2025;61(1):36-42.
In this article, we describe the course and diagnosis of pulmonary alveolar proteinosis (PAP) based on two cases from our practice. The first case is a 52-yearold woman, the second a 34-year-old man. Both referred patients were examined by a pulmonologist for interstitial lung disease, in the first case also with transition to pulmonary fibrosis. As part of the differential diagnosis, these patients were hospitalized at the NÚTPCHaHCH in Vyšné Hágy. Chest X-ray showed diffuse bilateral lung infiltrates, in the first patient locally confluent. Chest CT showed parenchymal involvement of the lungs with bilateral ground-glass opacities with thickened interlobular septa (crazy paving). Bronchoscopic examination was performed in both patients with bronchoalveolar lavage, which had a characteristic milky-glazed appearance. Videothoracoscopic lung biopsy was additionally indicated and histopathologically there were pulmonary alveolar proteinosis confirmed. Therapeutically, the patients underwent large volume lung lavage, with clinical condition improvement, including radiological findings improvement. We point out the basic pillars of the diagnosis of pulmonary alveolar proteinosis, which are the pattern of pulmonary involvement in the radiographic and CT (or HRCT) images, the characteristic appearance of the bronchoalveolar lavage fluid, and additionally also the histopathologic pattern of pulmonary involvement in this disease. We emphasize the need for centralized management of patients with lung diseases, which is particularly urgent in cases of rare diseases, where it provides rapid availability of all relevant diagnostic and therapeutic options, including large-volume lung lavage.
在本文中，我们基于我们临床实践中的两例病例描述肺泡蛋白沉积症（PAP）的病程及诊断。第一例病例为一名52岁女性，第二例为一名34岁男性。两位转诊患者均由肺科医生针对间质性肺疾病进行检查，第一例患者还出现了向肺纤维化的转变。作为鉴别诊断的一部分，这些患者在维什涅哈伊的NÚTPCHaHCH住院。胸部X线显示双侧肺部弥漫性浸润，第一例患者局部融合。胸部CT显示肺部实质受累，伴有双侧磨玻璃影及小叶间隔增厚（铺路石征）。两位患者均接受了支气管镜检查及支气管肺泡灌洗，灌洗液呈现出特征性的乳白光泽外观。此外还进行了电视胸腔镜肺活检，组织病理学检查确诊为肺泡蛋白沉积症。在治疗方面，患者接受了大容量肺灌洗，临床状况得到改善，包括影像学表现也有所改善。我们指出肺泡蛋白沉积症诊断的基本要点，即胸部X线和CT（或高分辨率CT）图像中的肺部受累模式、支气管肺泡灌洗液的特征性外观，以及该疾病肺部受累的组织病理学模式。我们强调对肺部疾病患者进行集中管理的必要性，这在罕见病病例中尤为迫切，因为这样能迅速提供所有相关的诊断和治疗选择，包括大容量肺灌洗。
