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

Abstract

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.

KEYWORDS:

• Tuberous sclerosis
• Lymphangioleiomyomatosis
• Oestrogen
• Contraception
• Combined hormonal contraceptives

ACKNOWLEDGEMENTS

This work was supported by Tuberöse Sklerose Deutschland e.V., which provided the database and forwarded the questionnaires. The authors are much indebted to Dr Klaus Schotten for verifing the statistical analysis of the data, and to Professor Dr Peter Nelson for undertaking a language edit of the manuscript.

Declaration of interest: The authors report no conflicts of interest. The authors alone are responsible for the content and the writing of the paper.