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Lung India Official publication of Indian Chest Society  
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ORIGINAL ARTICLE
Year : 2020  |  Volume : 37  |  Issue : 4  |  Page : 304-309

Management of primary pulmonary alveolar proteinosis: A multicentric experience


1 Department of Pulmonary, Critical Care and Sleep Medicine, Army Institute of Cardio- Thoracic Sciences (AICTS), Pune, Maharashtra, India
2 Department of Pulmonary, Critical Care and Sleep Medicine, Army Hospital (RR), Delhi Cantonment, Delhi, India
3 Department of Pulmonary Medicine, INHS Asvini, Mumbai, Maharashtra, India
4 Senior Consultant (Resp Medicine), Department of Pulmonary Medicine, Army College of Medical Sciences, Delhi Cantonment, Delhi, India
5 Department of Respiratory and Sleep Medicine, Artemis Hospital, Gurugram, Haryana, India
6 Department of Respiratory Medicine, Dr DY Patil Medical College, Hospital & Research Centre, Pimpri, Pune, Maharashtra, India
7 Department of Pulmonary Medicine, ILBS Hospital, New Delhi, India
8 Department of Pulmonary Medicine, Kokilaben Dhirubhai Ambani Hospital & Medical Research Institute, Mumbai, Maharashtra, India

Correspondence Address:
Dr. Vikas Marwah
Department of Pulmonary, Critical Care and Sleep Medicine, Army Institute of Cardio- Thoracic Sciences (AICTS), Pune, Maharashtra
India
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Source of Support: None, Conflict of Interest: None


DOI: 10.4103/lungindia.lungindia_401_19

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Background: Pulmonary alveolar proteinosis (PAP) is a rare disease characterized by alveolar accumulation of surfactant material with reduced lung function and resulting hypoxemia. It is characterized by a variable clinical course, and whole lung lavage (WLL) is the standard treatment. Herein, we report our multicentric experience of management of primary PAP. Materials and Methods: This retrospective study included patients with PAP managed at various armed forces respiratory centers from 2009 to 2019. The diagnosis of primary PAP was based on histopathologic confirmation on transbronchial lung biopsy or open lung biopsy and absence of causes of secondary PAP. We analyzed the response to WLL in these patients as well as the safety of the procedure. Results: During the above-specified period, ten patients with a diagnosis of PAP were admitted to various armed forces respiratory centers. The median age of the patients was 34.5 years (range 23–59); there were nine males (90%). The mean duration (± standard deviation) of symptoms was 10.8 (±2.70) months. For management, WLL was done for eight patients with a median volume of 23.5 L (range 18–45) per patient. All the patients showed significant symptomatic response as well as improvement in physiological parameters with no major complications. The median follow-up of all patients was 18 (range 5–44) months. Conclusions: WLL is a safe, effective therapy in an experienced setting in patients with PAP and provides long-lasting benefits.


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