Air travel and incidence of pneumothorax in lymphangioleiomyomatosis.

Details

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State: Public
Version: Final published version
Serval ID
serval:BIB_35699C0834DB
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Air travel and incidence of pneumothorax in lymphangioleiomyomatosis.
Journal
Orphanet journal of rare diseases
Author(s)
Gonano C., Pasquier J., Daccord C., Johnson S.R., Harari S., Leclerc V., Falconer L., Miano E., Cordier J.F., Cottin V., Lazor R.
ISSN
1750-1172 (Electronic)
ISSN-L
1750-1172
Publication state
Published
Issued date
13/12/2018
Peer-reviewed
Oui
Volume
13
Number
1
Pages
222
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: epublish
Abstract
Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease of women characterized by multiple lung cysts leading to respiratory insufficiency and frequent pneumothorax (PT). Air travel (AT) could increase the risk of PT in LAM through rupture of subpleural cysts induced by atmospheric pressure changes in aircraft cabin. To determine whether AT increases the risk of PT in LAM, we performed a retrospective survey of members of European LAM patient associations. A flight-related PT was defined as occurring ≤30 days after AT.
145 women reported 207 PT. In 128 patients with available data, the annual incidence of PT was 8% since the first symptoms of LAM and 5% since LAM diagnosis, compared to 0.006% in the general female population. Following surgical or chemical pleurodesis, the probability of remaining free of PT recurrence was respectively 82, 68, and 59% after 1, 5 and 10 years, as compared to only 55, 46 and 39% without pleurodesis (p = 0.026). 70 patients with available data performed 178 AT. 6 flight-related PT occurred in 5 patients. PT incidence since first symptoms of LAM was significantly higher ≤30 days after AT as compared to non-flight periods (22 versus 6%, risk ratio 3.58, confidence interval 1.40-7.45).
The incidence of PT in LAM is about 1000 times higher than in the general female population, and is further increased threefold after AT. Chemical or surgical pleurodesis partly reduces the risk of PT recurrence in LAM.
Keywords
Adult, Aged, Air Travel/statistics & numerical data, Female, Humans, Incidence, Lymphangioleiomyomatosis/epidemiology, Male, Middle Aged, Pleurodesis, Pneumothorax/epidemiology, Retrospective Studies, Air travel, Lymphangioleiomyomatosis, Pneumothorax
Pubmed
Web of science
Open Access
Yes
Create date
21/12/2018 12:17
Last modification date
20/08/2019 14:22
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