The emerging clinical picture of lymphangioleiomyomatosis

¹ Centre for Research in Women's Health, Canada
² University of Nottingham, United Kingdom

^* To whom correspondence should be addressed. E-mail: mmcohen{at}istar.ca.

Accepted 30 June 2005

	Abstract

Objective: To use two large patient registries to provide a comprehensive update of the clinical picture of Lymphangioleiomyomatosis (LAM).

Methods: Cross-sectional design included questions on 14 LAM symptoms, pneumothorax, tuberous sclerosis (TSC), date of diagnosis and pulmonary function tests (PFTs). Distribution of symptoms was compared across age, TSC, time since diagnosis, and PFTs using t-test or ANOVA, and regression; p<0.05.

Results: Response rate was 70.5% (n=328). Mean age at time of survey was 46.7 years. Mean age at diagnosis was 42.7 years for women diagnosed one year before the survey and 35.8 years for women diagnosed 10 years previously. Main symptoms were dyspnoea (74%), fatigue (72%), cough (47%) and chest pain (44%); younger patients (< 40 years) were less likely to report dyspnoea (p=0.02). TSC (n=51) patients were less likely to report dyspnoea (p=0.05) and 76.5% reported angiomyolipoma (p<0.0001) compared to sporadic LAM patients. 63.0% reported pneumothorax; these women were less likely to report dyspnoea or fatigue (p<0.05) than patients without pneumothorax. Mean PFTs % predicted were: FEV1, 67.4; FVC, 83.9, FEV1/FVC, 61.9%; TLC, 98; RV, 122; RV/TLC, 42%, and DLCOsb 65.3. Low FEV1 and DLCO were highly associated with dyspnoea (p< 0.0001) but not with fatigue or history of pneumothorax.

Conclusion: Previously considered a condition of women of childbearing age, more older women (50% without pneumothorax) are being diagnosed with LAM. LAM should be considered in women over 40 with unexplained dyspnoea. LAM patients with pneumothorax have less fatigue and less dyspnoea than those without pneumothorax. Fatigue has been overlooked as a symptom of LAM and appears across the spectrum of pulmonary function.

Keywords: dyspnea, lymphangioleiomyomatosis, pneumothorax, pulmonary function tests, tuberous sclerosis

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