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Fertility and pregnancy in women with primary adrenal insufficiency

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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.

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SB received a research grant from the regional agreement on medical training and clinical research (ALF) between Stockholm County Council and Karolinska Institutet.

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  1. Department of Endocrinology, Inflammation & Infection Theme, Karolinska University Hospital, 171 77 Stockholm, Sweden

    Sophie Bensing

  2. Department of Molecular Medicine and Surgery, Karolinska Institutet, 171 77, Stockholm, Sweden

    Sophie Bensing

  3. Department of Clinical and Biological Sciences and Division of Endocrinology, Diabetes and Metabolism-Department of Medical Sciences, University of Turin, 10123, Turin, Italy

    Roberta Giordano

  4. Section of Internal Medicine and Endocrinological and Metabolic Sciences, Department of Medicine, University of Perugia, 06129, Perugia, Italy

    Alberto Falorni

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Bensing, S., Giordano, R. & Falorni, A. Fertility and pregnancy in women with primary adrenal insufficiency. Endocrine 70, 211–217 (2020). https://doi.org/10.1007/s12020-020-02343-z

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