Fertility and pregnancy in women with primary adrenal insufficiency.

Primary adrenal insufficiency (PAI) occurs in ~1/5000-1/7000 individuals and is in most cases caused by autoimmune Addison's disease (AAD). Around 10-20% of women with AAD develop premature ovarian insufficiency (POI) before the age of 40 years. 21-Hydroxylase autoantibodies (21OHAb) are the best single immune marker to classify AAD among PAI patients and autoimmune POI in hypergonadotropic hypogonadic women. In AAD, detection of steroid-cell autoantibodies (StCA) predicts future development of POI. AAD-related autoimmune POI is characterized by a selective destruction of theca cells with preservation of primary follicles and granulosa cells of secondary and tertiary follicles. Women with AAD show reduced fertility and parity. Patients with well-managed disease are generally expected to have uneventful pregnancies with favorable outcome, but increased risk of maternal and neonatal complications has been reported. Hence, AAD pregnant women must be carefully monitored by skilled staff which is familiar with the disorder and specific attention must be given to the substitutive therapy.