Clinical Analysis of 6 Patients with Pulmonary Alveolar Proteinosis

Objective To investigate clinical manifestations,image feature and treatment of pulmonary alveolar proteinosis(PAP).Methods A retrospective analysis was conducted on 6 patients with PAP who were diagnosed by lung biopsy and pathological examination of bronchoalveolar lavage fluid(BALF).The changes of clinical symptoms,signs and image feature were studied.The effects of therapeutic whole lung lavage on PAP were evaluated.Results The PAP is clinically characterized by cough and progressive dyspnea.Physical examination is normal or reveals relatively minor and nonspecific pulmonary findings.Computed tomography scanning may show geographic air-space ground-glass opacities,crazy-paving pattern or interstitial fibrosis.The periodic acidic Schiff staining(PAS)in BALF was positive in 5 patients with PAP.After whole lung lavage,5 patients were relieved from clinical symptoms and image features was proved.Conclusion Bronchoalveolar lavage and lung biopsy were important diagnostic tool for assessment PAP.Computed tomography scan of the chest has a characteristic appearance of geographic and/or crazy-paving pattern.Therapeutic whole lung lavage is the principle option to treat patients with PAP.