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During normal gestation, adrenocorticotrophic hormone, corticotrophin\u2010releasing hormone, cortisol, and urinary free cortisol levels rise. Dexamethasone administration fails to fully suppress cortisol in pregnant women without CS. Localisation may be hindered by non\u2010suppressed adrenocorticotrophic hormone levels in a large proportion of those with adrenal CS; smaller corticotroph adenomas may go undetected as a result of a lack of contrast administration or the presence of pituitary hyperplasia; and inferior petrosal sinus sampling is not recommended given the risk of radiation and thrombosis. Yet, diagnosis is essential; active disease is associated with multiple insults to both maternal and foetal health, and those cured may normalise the risk of maternal\u2013foetal complications. The published literature consists mostly of case reports or small case series affected by publication bias, heterogeneous definitions of maternal or foetal outcomes or lack of detail on severity of hypercortisolism. Consequently, conclusive recommendations, or a standardised management approach for all, cannot be made. Management is highly individualised: the decision for surgery, medical control of hypercortisolism or adoption of a conservative approach is dependent on the timing of diagnosis (respective to stage of gestation), the ability to localise the tumour, severity of CS, pre\u2010existing maternal comorbidity, and, ultimately, patient choice. Close communication is a necessity with the patient placed at the centre of all decisions, with risks, benefits, and uncertainties around any investigation and management carefully discussed. Care should be delivered by an experienced, multidisciplinary team, with the resources and expertise available to manage such a rare and challenging condition during pregnancy.<\/jats:p>","DOI":"10.1111\/jne.13118","type":"journal-article","created":{"date-parts":[[2022,3,15]],"date-time":"2022-03-15T01:27:22Z","timestamp":1647307642000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":55,"title":["The diagnosis and management of Cushing's syndrome in pregnancy"],"prefix":"10.1111","volume":"34","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-9801-0458","authenticated-orcid":false,"given":"Ross","family":"Hamblin","sequence":"first","affiliation":[{"name":"Institute of Metabolism and Systems Research, College of Medical and Dental Sciences University of Birmingham  Birmingham UK"},{"name":"Centre for Endocrinology, Diabetes and Metabolism Birmingham Health Partners  Birmingham UK"},{"name":"Department of Endocrinology, Queen 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