Association of oestrogen-containing contraceptives with pulmonary lymphangioleiomyomatosis in women with tuberous sclerosis complex--findings from a survey.

[lymphangioleiomyomatosis]

OBJECTIVES About 30 -40 % of women with tuberous sclerosis complex (TSC ) develop pulmonary lymphangioleiomyomatosis (LAM ). Oestrogen seems to be involved in LAM pathogenesis and oestrogen-containing contraception should be avoided in women with known LAM . However , there is very little data on the use of contraceptives in TSC patients . METHODS We conducted a survey on the use of contraception and disease characteristics . The questionnaire was forwarded to all adult female TSC patients listed in the database of a German patient organisation . RESULTS Data from 39 such patients could be analysed . Of these , 15 were diagnosed with LAM . Twenty -five patients (65 %) confirmed current or past use of oestrogen-containing contraceptives . We found a suggestive correlation between the history of oestrogen-containing contraception , and LAM (Odds ratio : 6 .500 ; 95 % confidence interval : 1 .199 -35 .230 ). However , oestrogen use was not associated with LAM complications . CONCLUSIONS Based on our findings , oestrogen-containing contraceptives should be resorted to by these patients only with great caution , and avoided whenever possible .