Management of primary pulmonary alveolar proteinosis: A multicentric experience

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 re...

Full description

Saved in:
Bibliographic Details
Published in:Lung India 2020-07, Vol.37 (4), p.304-309
Main Authors: Marwah, Vikas, Katoch, C, Singh, Sarvinder, Handa, Ajay, Vardhan, Vasu, Rajput, A, Barthwal, M, Bhattacharyya, D, Rai, S
Format: Article
Language:English
Subjects:
Armed forces
Biopsy
Hypoxemia
Medical research
Original
Pulmonary alveolar proteinosis
Rare diseases
transbronchial lung cryobiopsy
whole lung lavage
Citations: Items that this one cites
Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary: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.
ISSN:0970-2113
0974-598X
DOI:10.4103/lungindia.lungindia_401_19