Gay Pierre, Wallaert Benoit, Nowak Stefan, Yserbyt Jonas, Anevlavis Stavros, Hermant Christophe, Lovis Alban, Menard Olivier, Maitre Bernard, Vandemoortele Thomas, Dutau Hervé, Briault Amandine, Bourdin Arnaud, Vergnon Jean-Michel, Froudarakis Marios E
Department of Pulmonology, University Hospital of Saint-Étienne, France.
Respiration. 2017;93(3):198-206. doi: 10.1159/000455179. Epub 2017 Jan 21.
New therapies have emerged in the treatment of pulmonary alveolar proteinosis (PAP) and, therefore, there is a real need to evaluate the efficacy of whole-lung lavage (WLL) in this rare disease.
The aim of this study was to assess the efficacy of WLL in patients with PAP.
We included 33 patients from 12 centers, which are members of the French-Speaking Thoracic Endoscopy Group, for analysis. Data collection concerned patients and disease characteristics, pulmonary function tests (PFTs) and technical information on the procedure.
The median age of the patients was 44 years (range 13-77). There were 23 (71.9%) patients with respiratory insufficiency at presentation. All patients underwent WLL by general anesthesia and selective lung ventilation, except 1 who underwent awake flexible bronchoscopy. We noted differences in the technique, as 12 (36.36%) patients had percussion during the procedure and only 4 (12.1%) patients underwent 2-lung lavage during 1 anesthesia. A median of 12 L was used to perform WLL (1.0-40 L). Complications occurred in 11 (33.3%) patients, and 18 (56.25%) of them relapsed in a median period of 16.9 months. No significant changes were found in any PFT parameters studied, except for PaO2, which was significantly improved by 6.375 mm Hg (p = 0.0213) after the procedure compared to before.
Although the application of the WLL technique was variable, overall, it significantly improved patients' short-term respiratory condition by improving PaO2. However, a long-term effect needs to be confirmed, as many of our patients relapsed.
肺泡蛋白沉积症（PAP）的治疗出现了新的疗法，因此，确实需要评估全肺灌洗（WLL）在这种罕见疾病中的疗效。
本研究的目的是评估WLL对PAP患者的疗效。
我们纳入了来自12个中心的33名患者进行分析，这些中心是法语区胸段内镜检查小组的成员。数据收集涉及患者和疾病特征、肺功能测试（PFT）以及该操作的技术信息。
患者的中位年龄为44岁（范围13 - 77岁）。有23名（71.9%）患者在就诊时存在呼吸功能不全。除1名患者接受清醒柔性支气管镜检查外，所有患者均在全身麻醉和选择性肺通气下接受WLL。我们注意到技术上存在差异，12名（36.36%）患者在操作过程中进行了叩击，只有4名（12.1%）患者在一次麻醉期间接受了双肺灌洗。进行WLL的灌洗量中位数为12升（1.0 - 40升）。11名（33.3%）患者出现并发症，其中18名（56.25%）在中位时间16.9个月后复发。除PaO2外，在所研究的任何PFT参数中均未发现显著变化，与操作前相比，操作后PaO2显著改善了6.375毫米汞柱（p = 0.0213）。
尽管WLL技术应用存在差异，但总体而言，它通过改善PaO2显著改善了患者的短期呼吸状况。然而，由于我们的许多患者复发，长期效果需要得到证实。
