Air travel and incidence of pneumothorax in lymphangioleiomyomatosis

Cynthia Gonano†,
Jérôme Pasquier†,
Cécile Daccord,
Simon R. Johnson,
Sergio Harari,
Violette Leclerc,
Lucy Falconer,
Eleonora Miano,
Jean-François Cordier,
Vincent CottinView ORCID ID profile and
Romain LazorEmail author View ORCID ID profile

†Contributed equally

Orphanet Journal of Rare Diseases201813:222

https://doi.org/10.1186/s13023-018-0964-6

Received: 1 October 2018
Accepted: 26 November 2018
Published: 13 December 2018

Abstract

Background

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.

Results

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).

Conclusions

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.