Long‑term follow‑up of whole lung lavage in patients with pulmonary alveolar proteinosis

Zhou,Xiaoyan; Lu,Guochu; Yu,Zhen; Gao,Fei; Bian,Tao

doi:10.3892/etm.2014.1788

Long‑term follow‑up of whole lung lavage in patients with pulmonary alveolar proteinosis

Authors:
- Xiaoyan Zhou
- Guochu Lu
- Zhen Yu
- Fei Gao
- Tao Bian
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Affiliations: Department of Respiratory Medicine, Wuxi People's Hospital Affiliated to Nanjing Medical University, Wuxi, Jiangsu 214023, P.R. China
Published online on: June 18, 2014 https://doi.org/10.3892/etm.2014.1788
Pages: 763-768

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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.

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