Abstract
Pulmonary alveolar proteinosis (PAP) is a rare disorder characterized by intra-alveolar accumulation of lipid and proteinaceous material. While a small subset of patients with PAP spontaneously improve or even undergo disease remission, the majority of patients develop persistent or progressive disease. Numerous therapies have been used to treat PAP over the years; however, at present, whole lung lavage (WLL) remains the gold standard treatment for PAP. In the present study, data were accumulated from a cohort of patients with PAP (n=11) between 2003 and 2011 at the Wuxi People’s Hospital Affiliated to Nanjing Medical University. The disease affected males and females with a ratio of 2.7:1 and all the males were current or previous smokers. The disease severity score (DSS) of the patients was mainly distributed in DSS 4 or DSS 5. All the patients underwent WLL at least once, with one patient undergoing WLLs twice and another patient three times. The clinical features, arterial blood gas and pulmonary function of the patients, were assessed prior to and following the lavage. WLL resulted in a significant improvement in symptoms, radiographic features, PaO2, D(A-a)O2 and DLCO in patients with PAP, while pulmonary ventilation function did not significantly improve. WLL appears to be an effective approach for the treatment of PAP and leads to an improvement in survival rate.
Highlights
Pulmonary alveolar proteinosis (PAP) is a rare diffuse pulmonary disease initially described by Rosen et al in 1958 [1]
Confirmation of diagnosis of PAP is achieved through using typical electron‐microscopic findings in sputum, lung washings and lung biopsy specimens, without which the condition is frequently mistaken for interstitial lung disease, sarcoidosis
The present study was conducted at the Wuxi People's Hospital Affiliated to Nanjing Medical University (Wuxi, Jiangsu, China) for the diagnosis and therapy of PAP in China
Summary
Pulmonary alveolar proteinosis (PAP) is a rare diffuse pulmonary disease initially described by Rosen et al in 1958 [1]. The clinical features of PAP include the accumulation of periodic acid‐Schiff (PAS)‐positive lipoproteinaceous material, predominantly phospholipid surfactants and surfactant. Patients with PAP may suffer from progressive dyspnea and cough, which may be accompanied by exacerbated hypoxia. Its course is variable, ranging from progressive deterioration to spontaneous improvement [2]. Radiographic analysis of the disease revealed ground‐glass opacities with multiple bilateral, irregular and dense inhomogeneous lesions, which have a ‘crazy‐paving’ pattern on computed tomography (CT) scans [2]. Confirmation of diagnosis of PAP is achieved through using typical electron‐microscopic findings in sputum, lung washings and lung biopsy specimens, without which the condition is frequently mistaken for interstitial lung disease, sarcoidosis
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