Pulmonary alveolar proteinosis in Taiwan

Cheng-Hao Chuang; Chih-Hung Cheng; Yu-Chen Tsai; Ming-Ju Tsai; Chau-Chyun Sheu; Inn-Wen Chong

doi:10.1016/j.jfma.2023.04.002

Pulmonary alveolar proteinosis in Taiwan

J Formos Med Assoc. 2023 Oct;122(10):1061-1068. doi: 10.1016/j.jfma.2023.04.002. Epub 2023 Apr 25.

Authors

Cheng-Hao Chuang¹, Chih-Hung Cheng², Yu-Chen Tsai³, Ming-Ju Tsai⁴, Chau-Chyun Sheu⁵, Inn-Wen Chong⁵

Affiliations

¹ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan; Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
² Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Department of Internal Medicine, Kaohsiung Municipal Siaogang Hospital, Kaohsiung, Taiwan.
³ Graduate Institute of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Department of Internal Medicine, Kaohsiung Municipal Ta-Tung Hospital, Kaohsiung, Taiwan.
⁴ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan; School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Department of Respiratory Care, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan. Electronic address: SiegfriedTsai@gmail.com.
⁵ Division of Pulmonary and Critical Care Medicine, Department of Internal Medicine, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan; School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan; Department of Respiratory Care, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.

PMID: 37105870
DOI: 10.1016/j.jfma.2023.04.002

Abstract

Background/purpose: Pulmonary alveolar proteinosis (PAP) is rare disease manifested as alveolar macrophage dysfunction and abnormal accumulation of surfactant protein in the alveoli. In this nationwide, population-based study, we investigated the epidemiology of PAP in Taiwan, and discovered the comorbidities and prognostic factors of PAP.

Methods: From the National Health Insurance Research Database (NHIRD), we obtained comprehensive information about all patients of PAP in Taiwan between 1995 and 2013. The incidence, baseline characteristics comorbidities, and prognostic factors of PAP were investigated.

Results: The annual incidence rate of PAP was around 0.79 (range: 0.49-1.17) patients per million people after 2000, and the prevalence rate was 7.96 patients per million people by the end of 2013. In total, 276 patients of PAP were identified, including 177 (64%) and 99 (36%) patients with primary and secondary PAP, respectively. The median age of diagnosis was 53.8 years. The median survival was 9.6 years after the initial PAP diagnosis, and the 5-year survival rate was 65.96%. Twenty (7%) patients received whole lung lavage (WLL) within three months after the diagnosis had significantly better survival compared to the others. Multivariable Cox regression analyses showed that elder age, secondary PAP, and malignancy were associated with poorer survival, while WLL within 3 months of diagnosis might greatly improve the survival.

Conclusion: We demonstrated the epidemiology of PAP in Taiwan, showing several poor prognostic factors and the potential effectiveness of WLL. Further prospective studies based on registry are warranted to improve the diagnosis and treatment of PAP.

Keywords: Pulmonary alveolar proteinosis; Statin; Whole lung lavage.

MeSH terms

Aged
Bronchoalveolar Lavage
Humans
Infant
Lung / pathology
Middle Aged
Prospective Studies
Pulmonary Alveolar Proteinosis* / diagnosis
Pulmonary Alveolar Proteinosis* / epidemiology
Pulmonary Alveolar Proteinosis* / therapy
Taiwan / epidemiology