Clinical features and outcomes of idiopathic pulmonary alveolar proteinosis in Korean population

Min Kwang Byun, Dong Soon Kim, Young Whan Kim, Man Pyo Chung, Jae Jeong Shim, Seung Ick Cha, Soo Taek Uh, Choon Sik Park, Sung Hwan Jeong, Yong Bum Park, Hong Lyeol Lee, Moo Suk Park

    Research output: Contribution to journalArticlepeer-review

    17 Citations (Scopus)

    Abstract

    Idiopathic pulmonary alveolar proteinosis (PAP) is a rare disorder in which lipoproteinaceous material accumulates within alveoli. There were few reports on Asian populations with idiopathic PAP. We retrospectively reviewed 38 patients with idiopathic PAP in Korea. We assessed clinical features, therapeutic efficacy and outcomes of whole lung lavage in patients with idiopathic PAP. The mean age at diagnosis was 52 yr. Eighty six percent of patients were symptomatic at diagnosis. Dyspnea and cough were the most common symptoms. Crackles were the most common physical examination finding. On pulmonary function test, a mild restrictive ventilatory defect was common, with a predicted mean forced vital capacity (FVC) of 77% and forced expiratory volume in one second (FEV1) of 84.6%. Diffusing capacity was disproportionately reduced at 67.7%. Arterial blood gas analysis revealed hypoxemia with a decreased PaO2 of 69.0 mmHg and an increased D(A-a)O2 of 34.2 mmHg. After whole lung lavage, PaO2, D(A-a)O2 and DLCO were significantly improved, but FVC and total lung capacity (TLC) were not different. This is the first multicenter study to analyze 38 Korean patients with idiopathic PAP. The clinical features and pulmonary parameters of Korean patients with idiopathic PAP are consistent with reports in other published studies. Whole lung lavage appears to be the most effective form of treatment.

    Original languageEnglish
    Pages (from-to)393-398
    Number of pages6
    JournalJournal of Korean medical science
    Volume25
    Issue number3
    DOIs
    Publication statusPublished - 2010 Mar

    Keywords

    • Irrigation
    • Koreans
    • Pulmonary alveolar proteinosis
    • Treatment outcome

    ASJC Scopus subject areas

    • Medicine(all)

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