Clinical Analysis Of 22 Cases With Pulmonary Alveolar Proteinosis

Objective: By analyzing the clinical characteristics,diagnosis,treatment and prognosis of patients with pulmonary alveolar proteinosis,we can improve the understanding of the disease.So as to guide the clinical practice,reduce the mortality of PAP patients,improve the efficacy and prognosis.Methods: Twenty-two patients with pulmonary alveolar proteinosis who were admitted to the Second Hospital of Hebei Medical University from June 2003 to June 2018 were analyzed retrospectively.Meanwhile,combined with the literatures related to PAP,the high-risk population,disease characteristics,laboratory examination,imaging and treatment methods were analyzed and summarized.Results: There were twenty-two patients,including fifteen males and seven females,with an average age of 48.5 ± 31.5 years old.Among them,smokers accounted for 50%,six patients had special occupational history in mining industry,chemical industry and cotton processing.All patients underwent subacute onset,with a history ranging from 21 days to 7 years,and the median duration are 3.5 years.The main clinical symptoms included cough,sputum and dyspnea,occasionally accompanied with hemoptysis,fever and others.The main manifestation of blood gas analysis was hypoxemia,and pulmonary function were diffusion impairment and restrictive ventilation dysfunction.Some patients with long-term smoking history suffered from obstructive ventilation dysfunction.The CT manifestations of the lung were diffusely distributed consolidation shadow,ground-glass opacity,nodules,etc.PAS staining of bronchoalveolar lavage fluid showed positive.eight patients were given symptomatic treatment,of which seven cases were improved;14 patients underwent whole lung lavage,ten of them were significantly improved after one whole lung lavage,one patient had a recurrence due to pregnancy,because of poor effect of whole lung lavage,three patients were treated with whole lung lavage combined with granulocyte macrophage-colony stimulating factor,and one patient was significantly improved.Conclusions:1.Pulmonary alveolar proteinosis can be diagnosed by combining results of lung CT examination and PAS staining of lavage.2.The clinical manifestations of pulmonary alveolar proteinosis have no specific characteristics.Suspected PAP patients in clinical practice should be diagnosed by bronchoalveolar lavage as early as possible.3.Patients with obvious clinical symptoms can be treated by whole lung lavage therapy.Granulocyte macrophage-colony stimulating factor can be used for patients with poor efficacy.