Abstract
Pulmonary alveolar proteinosis is such an extremely rare disease in Korea, that
only a few cases have been reported. Meanwhile five cases were experienced at
Seoul National University Hospital over ten years since 1987. We summarized the
clinical characteristics and courses of them. Seven cases reported in the
literature were included to add data about clinical characteristics and courses
although only a few case reports mentioned patient's course. Middle aged male
patients were mainly affected. No association with particular environmental or
occupational exposure was identified. Dyspnea on exertion was the main symptom.
Bilateral crackles were consistent, and bilateral parahilar hazy infiltrations
on plain chest radiograph and ground glass opacity on high-resolution CT were
characteristic. Superimposed infection was not identified in any patient at the
time of diagnosis. Decreased diffusing capacity and hypoxia were present in
almost every case. Whole lung lavage proved to be an effective therapeutic
measure. The response to treatment was good. Long-term course of the disease,
e.g. recurrence rate, is not yet known.