PULMONARY ALVEOLAR PROTEINOSIS - CLINICAL-FEATURES AND OUTCOMES

Introduction: Pulmonary alveolar proteinosis (PAP) is a rare disease o f unknown etiology and variable natural history, To date, the largest series from a single institution has consisted of 68 individuals, To e xtend the understanding of the clinical features and natural history o f PAP, we present a series of 24 patients with PAP from a single insti tution. Methods: Patients with PAP were identified by a review of The Cleveland Clinic Foundation discharge database from 1965 to 1995. Afte r identification, a chart review of the 24 selected patients was condu cted. Charts were abstracted for historical, diagnostic, and therapeut ic features. Diagnosis was confirmed. by a review of the lung biopsy, where it was available, by a lung pathologist. Follow-up by telephone was performed with 19 patients. Results: Most patients (70%) were male , and smoking was common (75%; mean pack-years, 29) in the group. The mean age at initial evaluation was 43 years. Presenting symptoms were as follows: 19 patients (79%) reported dyspnea, 19 patients (79%) repo rted a cough, 4 patients (17%) reported hemoptysis, and 3 patients (13 %) reported chest pain. The earliest available spirometry after presen tation of symptoms showed a prediction of mean FEV1, of 74% (range, 45 to 99%) and a prediction of mean FVC of 76% (range, 41 to 99%). The d iagnosis of PAP nas established by transbronchial biopsy alone in 7 pa tients (29%) and by open-lung biopsy alone in 17 patients (71%). Whole lung lavage was deemed necessary in 13 patients (54%); 3 patients und erwent lavage of one lung only, and 10 patients underwent bilateral wh ole lung lavage. Whole lung lavage was required only once in 46% of pa tients, and from two to four times in the remainder of patients. Durin g the follow-up period (mean length of follow-up, 8.5 years; range, 5 months to 21 years), 25% of the patients died, but none as a result of sequelae of PAP. Half of the survivors reported persistent symptoms. Conclusions: In this series, which represents one of the largest singl e institutional experiences with PAP reported, the clinical features a re largely consistent with previously reported cases. However, contrar y to early reported experiences in which open-lung biopsy was frequent ly required to establish PAP and in which whole lung lavage was needed , transbronchial biopsy established the diagnosis in 29% of patients a nd whole lung lavage could be deferred in 46% of patients.