Clinical features and outcomes of idiopathic pulmonary alveolar proteinosis in Korean population.
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 (FEV(1)) 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 DL(CO) 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.
Byun
Min Kwang
MK
Division of Pulmonary Medicine, Department of Internal Medicine, Yonsei University, College of Medicine, Yonsei University Health System, Seoul, Korea.
Kim
Dong Soon
DS
Kim
Young Whan
YW
Chung
Man Pyo
MP
Shim
Jae Jeong
JJ
Cha
Seung Ick
SI
Uh
Soo-Taek
ST
Park
Choon Sik
CS
Jeong
Sung Hwan
SH
Park
Yong Bum
YB
Lee
Hong Lyeol
HL
Park
Moo Suk
MS
D000328:Adult; D001706:Biopsy; D018893:Bronchoalveolar Lavage; D005260:Female; D006801:Humans; D007723:Korea; D008297:Male; D008875:Middle Aged; D011649:Pulmonary Alveolar Proteinosis / Q000175:diagnosis / Q000473:pathology / Q000503:physiopathology* / Q000628:therapy*; D012129:Respiratory Function Tests; D012189:Retrospective Studies; D015996:Survival Rate; D016896:Treatment Outcome