Air travel and incidence of pneumothorax in lymphangioleiomyomatosis
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(2018) 13:222
RESEARCH
Open Access
Air travel and incidence of pneumothorax in lymphangioleiomyomatosis Cynthia Gonano1†, Jérôme Pasquier2†, Cécile Daccord3, Simon R. Johnson4, Sergio Harari5, Violette Leclerc6, Lucy Falconer7, Eleonora Miano8, Jean-François Cordier9, Vincent Cottin9 and Romain Lazor3*
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. Keywords: Lymphangioleiomyomatosis, Incidence, Pneumothorax, Pleurodesis, Air travel
Background Pulmonary lymphangioleiomyomatosis (LAM) is a rare disease affecting almost exclusively women in their reproductive age. It is characterised by the proliferation of abnormal smooth muscle-like cells (LAM cells) in the lungs and lymphatic system, and is considered as a low-grade metastasizing neoplasm [1–5]. In the lungs, LAM cell proliferation leads to the development of multiple thin-walled cysts and progressive destruction of the parenchyma resulting in dyspnea, obstructive ventilatory defect, reduced carbon monoxide transfer factor, and hypoxemia [6–10]. Another typical feature of pulmonary LAM is the occurrence of pneumothorax (PT), which affects more than half of patients during disease course with frequent relapses [6–9]. * Correspondence: [email protected] † Cynthia Gonano and Jérôme Pasquier contributed equally to this work. 3 Service de pneumologie, Centre hospitalier universitaire vaudois, PMU BU44.07, Rue du Bugnon 44, 1011 Lausanne, Switzerland Full list of author information is available at the end of the article
Air travel (AT) is a matter of concern in patients with LAM [11]. During commercial flights, the cruising altitude varie
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