ESPE Yearbook of Paediatric Endocrinology

Endocrine. 2024; 86(3): 1156-1170. PMID: 39277840 doi: 10.1007/s12020-024-04023-8. https://pubmed.ncbi.nlm.nih.gov/39277840/

Brief summary: This systematic review and meta-analysis evaluated the impact of maternal primary adrenal insufficiency (PAI) during pregnancy, due to either Addison disease (AD) or congenital adrenal hyperplasia (CAH), with data from PubMed/Medline, Cochrane/CENTRAL, and Google Scholar. A total of 31 studies were included for qualitative analysis and 11 for quantitative analysis.

Comment: Women with primary adrenal insufficiency (PAI) face an increased risk of negative pregnancy and fetal outcomes, including higher rates of miscarriage, preterm birth, and small for gestational age (SGA) babies (1). There is also an increased risk of adrenal crises during pregnancy, and while maternal mortality is uncommon, suboptimal management can be fatal (2).

This systematic review and meta-analysis found a higher prevalence of pregnancies with negative outcome (spontaneous abortion, preterm birth) and of negative fetal outcome (SGA) in women with either AD or CAH, compared with control pregnancies. Of note, the meta-analysis showed a mean prevalence of spontaneous abortion of 18% (18% and 17% in women with AD or CAH, respectively). The mean prevalence of preterm birth was 11% (13% and 9% in women with AD or CAH, respectively). The mean prevalence of SGA neonates was 8% (5% and 10% in women with AD or CAH, respectively). Mean fetal birth weight was within normal range in all women with PAI, as well as in women with AD or CAH.

Pregnancy with PAI can be successful, but it requires careful management and monitoring to mitigate the increased risks of adverse outcomes (3). A multidisciplinary approach, including specialized endocrine and obstetric care, is essential for optimizing both maternal and fetal well-being (3).

References: 1. Cauldwell M, Steer PJ, Ahsan M, Ali A, Ashiq S, Ashworth R, Basha D, Chong H, Corbett GA, Dunne F, Hill A, Gajewska-Knapik K, Jakes A, McLaren D, Kinsella T, Lee T, Levy M, MacKiliop L, McAuliffe FM, Mohan A, Mumby C, Nana M, Napier C, Neuberger F, Newman C, Oosterhouse T, Shard A, Shehata H, Stocker L, Tomlinson JW, Beck A, Vaidya B, Wiles K, Williamson C, Zollner J, Ward E, Turner HE. Pregnancy Outcomes in Women With Primary Adrenal Insufficiency: Data From a Multicentre Cohort Study. BJOG. 2025;132(8):1122-1129.2. Gardella B, Gritti A, Scatigno AL, Gallotti AMC, Perotti F and Dominoni M. Adrenal crisis during pregnancy: Case report and obstetric perspective. Front. Med. 2022; 9:891101.3. Ahmed M, Bashir M, Okunoye GO, Konje JC. Adrenal disease and pregnancy: an overview. The Obstetrician &Gynaecologist 2021; 23: 265–277.