J Korean Med Sci.  1999 Apr;14(2):159-164. 10.3346/jkms.1999.14.2.159.

The clinical characteristics of pulmonary alveolar proteinosis: experience at Seoul National University Hospital, and review of the literature

• Kim G
• Lee SJ
• Lee HP
• Yoo CG
• Han SK
• Shim YS
• Kim YW

• KMID: 648074
• DOI: http://doi.org/10.3346/jkms.1999.14.2.159

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.

Keyword

Pulmonary alveolar proteinosis

MeSH Terms

Adult
Age Distribution
Aged
Female
Hospitals, Public*
Hospitals, University*
Human
Korea
Male
Middle Age
Pulmonary Alveolar Proteinosis/therapy
Pulmonary Alveolar Proteinosis/radiography
Pulmonary Alveolar Proteinosis/physiopathology*
Pulmonary Alveolar Proteinosis/diagnosis
Sex Distribution
Smoking/adverse effects