Adrenal insufficiency in pregnancy: challenging issues in diagnosis and management

Abstract

Adrenal insufficiency (AI) in pregnancy is relatively rare, but it is associated with significant maternal and fetal morbidity and mortality if untreated during gestation or in the puerperium. Hence, timely diagnosis and decisive treatment by the clinician are critical. However, due to pregnancy-induced metabolic and endocrine changes and the resemblance of symptomatology of AI to those of pregnancy, the diagnosis is often difficult to recognize and challenging to confirm. Normal pregnancy is a state of glucocorticoid excess particularly in the latter stages, and normative values for serum cortisol levels are not well-established. Furthermore, testing the hypothalamic-pituitary-adrenal axis using validated stimulation tests during pregnancy are lacking. Therefore, it is the aim of the present review to discuss and to summarize the current knowledge, focussing on the challenges in recognizing AI in pregnancy and interpreting the diagnostic tests, and to propose a clinical approach for optimizing the management of AI in women diagnosed before or during pregnancy.

Unilateral adrenal infarction in pregnancy

“…This results in an increase in pituitary ACTH production to increase free cortisol levels, which rise into the third trimester. The diagnosis of adrenal insufficiency requires: (1) demonstrating low serum cortisol levels; (2) determining if the cortisol deficiency is dependent on or independent of an ACTH deficiency and assessing mineralocorticoid secretion in patients without ACTH deficiency; and (3) finding a treatable cause of the primary disorder 12. When the clinical suspicion for adrenal crisis is high, samples for serum cortisol and ACTH levels can be obtained.…”

MANAGEMENT OF ENDOCRINE DISEASE: Fertility, pregnancy and lactation in women with adrenal insufficiency

“…In fact, prior to the introduction of glucocorticoid replacement therapy, Addison's disease was associated with a high maternal mortality (35-45%), so that women were strongly discouraged from becoming pregnant (1,2,3,4,5,6,7,8,9,10,11). Modern glucocorticoid replacement therapy and improved obstetric care have both contributed immensely in reducing maternal as well as fetal morbidity and mortality.…”

“…Additionally, it is responsible for various changes in the maternal hypothalamic pituitary adrenal (HPA) axis leading to a state of physiological hypercortisolism in pregnancy (1,2,3,4,5,6,7,8,9,10,11,12).…”

Pregnancy, Diabetes Insipidus and Adrenal Insufficiency after Surgical Treatment in Clinically Non-functioning Pituitary Adenoma: A Case Report

“…Corticotrophic insufficiency leads to increased morbidity and maternal-fetal mortality, with an increased prevalence of symptoms such as hyperemesis of pregnancy, postural hypotension and fatigue beyond the first trimester, as well as a risk of adrenal crisis due to the reduction of cortisol in the mother [10]. For the fetus, there is the increased incidence of oligohydramnios, low birth weight and intrauterine death [10].…”

“…For the fetus, there is the increased incidence of oligohydramnios, low birth weight and intrauterine death [10]. During normal pregnancies, there is a physiological increase in total and free cortisol values.…”