Background: Few reports exist regarding the long-term clinical course of idiopathic pulmonary alveolar proteinosis.
Purpose And Methods: We retrospectively studied the clinical courses of 8 patients. The patients were 4 men and 4 women aged 48.4 +/- 14.0 years (mean +/- SD) with idiopathic pulmonary alveolar proteinosis followed up for a minimum of more than 4 years. Mean follow-up was 13 years and 6 months (4-22 years and 9 months).
Results: There were 15 instances of whole-lung lavage and/or bronchoalveolar lavage in 7 patients, and lavage was effective 40% of cases. Spontaneous improvement occurred 16 times and exacerbation occurred 7 times in 8 patients. On chest X-ray films, the shadows disappeared in 5 of 8 (62.5%) patients, and in 4 of 5 patients, shadows disappeared 5 or more years after diagnosis of pulmonary alveolar proteinosis. Pulmonary shadows have not disappeared in the remaining 3 patients at the time of writing but have shown improvement compared with first presentation. There were no deaths.
Conclusion: As indicated in previous reports, the long-term outcome of idiopathic pulmonary alveolar proteinosis appears to be good.
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