Extensive expertise in endocrinology: adrenal crisis in assisted reproduction and pregnancy

Abstract

Appropriate management of adrenal insufficiency in pregnancy is challenging due to the rarity of both primary, secondary and tertiary forms of the disease as well as the lack of evidence-based recommendations to guide clinicians to glucocorticoid and sometimes also mineralocorticoid dosage adjustments. Debut of adrenal insufficiency during pregnancy requires immediate diagnosis as it can lead to adrenal crisis, intrauterine growth restriction and foetal demise. Diagnosis is difficult due to overlap of symptoms of adrenal insufficiency and its crisis with those of pregnancy. Adrenal insufficiency in stable replacement treatment needs careful monitoring during pregnancy to adapt to the physiological changes in the requirement of the adrenal hormones. This is hampered because the diagnostic threshold of most adrenocortical hormones is not applicable during pregnancy. The frequent use of assisted reproduction technology with controlled ovarian hyperstimulation in these patient groups with disease induced low fertility has created an unrecognised risk of adrenal crises due to accelerated oestrogen stimulation with increased risk of even be life-threatening complications for both the woman and foetus. The area needs consensus recommendations between gynaecologists and endocrinologists in tertiary referral centres to alleviate such increased gestational risk. Patient and partner education, use of the EU emergency card for management of adrenal crises can also contribute to better pregnancy outcomes. There is a strong need of more research on e.g. improvement of glucocorticoid replacement as well as crisis management treatment, and biomarkers for treatment optimisation in this field, which suffers from the rare nature of the diseases and poor funding.