Primary adrenal insufficiency in pregnancy: A review article

Abstract

Background:
Primary adrenal insufficiency is a rare disease in the general population with an estimated
incidence of approximately 144 cases per million.1,3 Congenital adrenal hyperplasia (CAH)
and autoimmune adrenalitis (AA) are the two most common causes for primary adrenal
insufficiency in general.1
Primary adrenal insufficiency can be congenital or acquired.2 Congenital types include
congenital adrenal hyperplasia (CAH), familial glucocorticoid deficiency (FGD) and
congenital adrenal hypoplasia.2Autoimmune adrenalitis and tuberculosis account for the
majority of acquired primary adrenal insufficiency.2 In AA, there is autoimmune mediated
destruction of the adrenal cortex resulting in cortisol, mineralocorticoid and androgen
deficiency.1Polyendocrine syndrome (APS) is when this autoimmune process extend to involve
the pancreas causing type 1 diabetes and the thyroid gland causing autoimmune thyroid
disease.1
Addison disease is the term used to describe acquired primary adrenal insufficiency2
a disease that is more prevalent in women than men with a peak incidence in third and fourth
decades of life3
. This makes good understanding of this disease during pregnancy crucial. The
estimated prevalence of adrenal insufficiency during pregnancy is 10 per 100 000.3 This
includes all cases of adrenal insufficiency, primary or secondary congenital or acquired.
As mentioned, Adrenal disorders in pregnancy are not common, the autoimmune nature
of the disease leading to chronic anovulation and impaired infertility is one of the reasons 13
.
timely diagnosis is imperative because these disorders can lead to significant maternal and
fetal morbidity. Making the diagnosis poses a challenge to the clinician because the fetalplacental unit alters the maternal endocrine metabolism and hormonal feedback mechanisms.
Pregnancy and its hypermetabolic state may alter the manifestation of disease, making the
diagnosis difficult.