PURPOSE: Background:Lymphangioleiomyomatosis (LAM) is a rare cystic lung disease that predominantly affects young women. Pneumothorax commonly occurs repeatedly and multiple cysts in the lungs are considered to be at risk of pneumothorax during air travel. Therefore patients have impaired quality of life. Objectives: To evaluate the features of pneumothorax and risk of air travel in lymphangioleiomyomatosis.
METHODS: We prepared questionnaire about pneumothorax and asked LAM patients who visited at Juntendo hospital from April 2010 to May 2011. We analyzed medical information, history and treatment of pneumothorax, background information relating incidence of pneumothorax and risk of air travel.
RESULTS: One hundred and four LAM patients agreed to fill out the questionnaire. Their mean age at the time of survey was 42.1 years old (range 22 - 64) and prevalence of TSC was 10.6% (11 patients). Fifty eight percent (61/104) of respondents had a history of radiographically documented pneumothorax. The mean age at the first pneumothorax was 32.8 years (range 20 - 46). Pneumothrax recurred in 75% (41/61) and average number of pneumothorax per patient was 5.1 (range 1- 41 times). Sixty one percent (37/61) of patients with history of pneumothorax reported that pneumothorax occurred during stressful environment (either mentally or physically, or both) and 72% (44/61) of patients reported a lifestyle modification due to fear of a pneumothorax (e.g. air travel, heavy exercise). Among respondents, 98 patients (94%) reported that they had flown by aircraft for a total of 2,212 flights (1,854 flights before diagnosis and 358 after diagnosis). Six respondents had a symptom during air travel (total 10 adverse events, most of the symptoms ware tightness of chest). But there was no evidence about occurrence of pneumothorax.
CONCLUSIONS: Pneumothorax complicated frequently in LAM and recurred in 75% of patients. It occurred commonly during stressful environment rather than strenuous activity.
CLINICAL IMPLICATIONS: There is a very low risk of pneumothorax following air travel in our study although many LAM patients tend to restrict air travel after the diagnosis of LAM.
DISCLOSURE: The following authors have nothing to disclose: Yoshito Hoshika, Hideyuki Kataoka, Masatoshi Kurihara, Katsutoshi Ando, Kuniaki Seyama, Kazuhisa Takahashi
No Product/Research Disclosure Information