open access

Vol 86, No 1 (2018)
ORIGINAL PAPERS
Published online: 2018-02-28
Submitted: 2017-07-03
Accepted: 2018-01-26
Get Citation

An eleven-year retrospective cross-sectional study on pulmonary alveolar proteinosis

Arda Kiani, Tahereh Parsa, Parisa Adimi Naghan, Hervé Dutau, Fatemeh Razavi, Behrooz Farzanegan, Mahsa Pourabdollah Tootkaboni, Atefeh Abedini
DOI: 10.5603/ARM.2018.0003
·
Pubmed: 29490416
·
Adv Respir Med 2018;86(1):7-12.

open access

Vol 86, No 1 (2018)
ORIGINAL PAPERS
Published online: 2018-02-28
Submitted: 2017-07-03
Accepted: 2018-01-26

Abstract

Introduction: Pulmonary alveolar proteinosis (PAP) is a rare disease in the field of pulmonary medicine. The efficacy of whole-lung
lavage (WLL) as the treatment of PAP had never been evaluated in the Iranian population. Therefore, there is a real need to investigate
the characteristics of PAP and also to evaluate the efficacy of WLL in this rare disease. The study aimed to investigate
demographic features, clinical presentation and treatment outcomes of the disease in Iranian PAP patients.

Material and methods: Data of 45 patients with definite diagnosis of PAP, who had regular follow-ups from March 2004 to March
2015 at an Iranian referral respiratory hospital, were collected. Whole-lung lavages (WLL) efficacy was assessed by comparing
spirometric, arterial blood gas parameters and six-minute walk test (6MWT) results before and after all lavages.

Results: Mean age at diagnosis of disease was 30.33 ± 14.56 years. Four patients (8.8%) reported non-massive hemoptysis and three
subjects (6.6%) had concomitant pulmonary tuberculosis. In 71.1% of cases, transbronchial lung biopsy and bronchoalveolar lavage were
sufficient for diagnosis. Spirometric results and arterial blood gas parameters and 6MWD improved significantly after all the lavages. Four
patients (8.8%) died because of respiratory failure. The only variable capable of predicting treatment failure was the history of hemoptysis.

Conclusion: The study revealed sufficiency of WLL as the PAP patients’ treatment. Also hemoptysis was found to be the independent
factor that can predict treatment failure.

Abstract

Introduction: Pulmonary alveolar proteinosis (PAP) is a rare disease in the field of pulmonary medicine. The efficacy of whole-lung
lavage (WLL) as the treatment of PAP had never been evaluated in the Iranian population. Therefore, there is a real need to investigate
the characteristics of PAP and also to evaluate the efficacy of WLL in this rare disease. The study aimed to investigate
demographic features, clinical presentation and treatment outcomes of the disease in Iranian PAP patients.

Material and methods: Data of 45 patients with definite diagnosis of PAP, who had regular follow-ups from March 2004 to March
2015 at an Iranian referral respiratory hospital, were collected. Whole-lung lavages (WLL) efficacy was assessed by comparing
spirometric, arterial blood gas parameters and six-minute walk test (6MWT) results before and after all lavages.

Results: Mean age at diagnosis of disease was 30.33 ± 14.56 years. Four patients (8.8%) reported non-massive hemoptysis and three
subjects (6.6%) had concomitant pulmonary tuberculosis. In 71.1% of cases, transbronchial lung biopsy and bronchoalveolar lavage were
sufficient for diagnosis. Spirometric results and arterial blood gas parameters and 6MWD improved significantly after all the lavages. Four
patients (8.8%) died because of respiratory failure. The only variable capable of predicting treatment failure was the history of hemoptysis.

Conclusion: The study revealed sufficiency of WLL as the PAP patients’ treatment. Also hemoptysis was found to be the independent
factor that can predict treatment failure.

Get Citation

Keywords

bronchoalveolar lavage (D018893), pulmonary alveolar proteinosis (D011649), whole-lung lavage

About this article
Title

An eleven-year retrospective cross-sectional study on pulmonary alveolar proteinosis

Journal

Advances in Respiratory Medicine

Issue

Vol 86, No 1 (2018)

Pages

7-12

Published online

2018-02-28

DOI

10.5603/ARM.2018.0003

Pubmed

29490416

Bibliographic record

Adv Respir Med 2018;86(1):7-12.

Keywords

bronchoalveolar lavage (D018893)
pulmonary alveolar proteinosis (D011649)
whole-lung lavage

Authors

Arda Kiani
Tahereh Parsa
Parisa Adimi Naghan
Hervé Dutau
Fatemeh Razavi
Behrooz Farzanegan
Mahsa Pourabdollah Tootkaboni
Atefeh Abedini

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