Background: Lymphangioleiomyomatosis (LAM) is a rare pulmonary disease of women of unknown etiology. Diffuse cystic changes associated with multifocal clusters of smooth muscle-like cells (LAM cells) replace normal lung parenchyma, leading to progressive loss of lung function. Reports of exacerbations of LAM with the use of exogenous estrogens and during pregnancy suggest an association between hormones and clinical deterioration.
Objectives: To conduct the largest case series to date in order to characterize the demographics of a living cohort of patients with LAM. To evaluate the effect of oral contraceptive pill (OCPs) use on the age at onset of symptoms in a large population of women with LAM.
Methods: The study population was drawn from the University of Miami (UM) LAM database consisting of 91 living women previously diagnosed with LAM by a physician. A self-administered questionnaire was mailed to the participants.
Results: The final response rate was 91.2% (83 of 91), and the study participants were 96.4% white, 1.2% Asian, 1.2% African American, and 1.2% American Indian; 68.4% identified their ethnicity as European and 14.5% as Jewish. Of the women surveyed, 73 were still menstruating at the time of LAM diagnosis. Among these women, the rate of use of OCPs was approximately 27%, similar to the rate in the general population. There was a significant difference between the age at onset of symptoms in women using OCPs vs. women not using OCPs (29.2 +/- 4.7 vs. 32.9 +/- 8.0, p = 0.0397).
Conclusions: The present study suggests that OCPs may serve as catalysts to promote an earlier occurrence of LAM.