• Ning Zhang, Zhilong Jiang, and Changzhou Shao.
• Department of Pulmonary Medicine, Xiamen Branch, Zhongshan Hospital, Fudan University, Xiamen 361000, China; Shanghai Institute of Respiratory Diseases, Department of Pulmonary Medicine, Zhongshan Hospital, Fudan University, Shanghai 200032, China.
• Med Clin (Barc). 2020 Aug 24.

IntroductionPulmonary alveolar proteinosis (PAP) is a rare lung disease, characterized by abnormal alveolar accumulation of enlarged foamy macrophages and periodic acid-Schiff (PAS)-positive materials. Knowledge of the disease characteristics is still lacking.ObjectiveTo help clinicians gain a better understanding of this rare disease.MethodsWe undertook a retrospective analysis of 14 adult patients with PAP, treated in Zhongshan Hospital, Fudan University.ResultsSerum lactate dehydrogenase (LDH) was correlated with the arterial partial pressure of oxygen (PaO2) and diffusion capacity for carbon monoxide (DLCO). Transbronchial lung biopsy (TBLB) established a definitive diagnosis for a positive rate of 100%. The patients underwent whole lung lavage (WLL) and exhibited varying degrees of remission. The patients with mild symptoms received only supportive care and observation, and remained stable during follow-up.ConclusionLDH may correlate with disease severity. Bronchoscopy is sufficiently sensitive for a definite diagnosis. Conventional bilateral whole lung lavage proved a reliable treatment for indicated patients, but selective unilateral lung lavage or observation may be a rational choice in certain patients.Copyright © 2020 Elsevier España, S.L.U. All rights reserved.

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