Fertility and pregnancy in women with primary adrenal insufficiency
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MINI REVIEW
Fertility and pregnancy in women with primary adrenal insufficiency Sophie Bensing1,2 Roberta Giordano3 Alberto Falorni ●
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Received: 18 December 2019 / Accepted: 6 May 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract 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. Keywords Adrenal insufficiency Autoantibodies Pregnancy Premature ovarian insufficiency ●
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Introduction Primary adrenal insufficiency (PAI) results from the destruction, surgical removal, or impaired function of the adrenal cortex [1]. Autoimmune Addison’s disease (AAD) is the major cause of PAI [1]. The prevalence of PAI is 1/ 5000–1/7000 in the general population [2–5], with an incidence of 6 cases/million/year [3]. Over 80% patients with AAD present with clinical or biochemical signs of another autoimmune disease, the most frequent being thyroid autoimmune diseases, type 1 diabetes mellitus (T1DM), vitiligo, chronic atrophic gastritis,
* Alberto Falorni [email protected] 1
Department of Endocrinology, Inflammation & Infection Theme, Karolinska University Hospital, 171 77 Stockholm, Sweden
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Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 77 Stockholm, Sweden
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Department of Clinical and Biological Sciences and Division of Endocrinology, Diabetes and Metabolism-Department of Medical Sciences, University of Turin, 10123 Turin, Italy
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Section of Internal Medicine and Endocrinological and Metabolic Sciences, Department of Medicine, University of Perugia, 06129 Perugia, Italy
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and premature ovarian insufficiency (POI) [6, 7]. Hence, AAD is a major component of autoimmune polyendocrine syndrome type 1 (APS1)—along with chronic candidiasis and hypoparathyroidism—and type 2 (APS2) [8]. Adrenal autoimmunity tends to develop more frequently in females than in males and specific attention to women’s fertility and fecundity must be taken in the management of patients with AAD.
Autoimmune POI Approximately 10–
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