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A Journal on Endocrine System Diseases

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Minerva Endocrinologica 2018 Feb 13

DOI: 10.23736/S0391-1977.18.02803-1


language: English

Cushing's disease: major difficulties in diagnosis and management during pregnancy

Francisca CAIMARI 1, 2, Rosa CORCOY 1, 2, 3, 5, Susan M. WEBB 1, 2, 4, 5

1 Endocrinology/Medicine Department, Hospital Sant Pau, Barcelona, Spain; 2 Universitat Autònoma de Barcelona, Barcelona, Spain; 3 CIBER Bioengineering, Biomaterials and Nanotechnology, Instituto de Salud Carlos III, Madrid, Spain; 4 Centro de Investigación Biomédica en Red de Enfermedades Raras (CIBERER, Unidad 747), IIB-Sant Pau, Instituto de Salud Carlos III, Barcelona, Spain; 5 Teaching Unit Hospital Sant Pau, UAB, Barcelona, Spain


Pregnancy in women with a diagnosis of Cushing’ syndrome (CS) is an extremely rare event and its diagnosis and treatment are a real medical challenge. During pregnancy, the hypothalamus-pituitary-adrenal axis undergoes major changes leading to a significant increase in plasma cortisol levels throughout gestation. The difficulties in diagnosis are related to the resemblance of symptoms of CS and those of pregnancy, and to the complex interpretation of the screening tests. Moreover, the diagnostic work up in the postnatal period may be difficult in the first weeks postpartum. Importantly, the aetiology of CS in pregnancy differs from non-pregnant status. In pregnancy, the adrenal origin is the most frequent in up to 60% of the cases, in contrast to ACTH-secreting corticotroph adenomas of the pituitary gland, which account for 70% of the cases outside pregnancy. Nevertheless, maternal and foetal outcomes are severely affected in the context of CS whichever the aetiology is, with high rates of maternal and foetal morbimortality, and with a rate of overall foetal loss of about 25% of the pregnancies. There is no consensus as to the most effective treatment in these circumstances in terms of improving maternal and foetal outcomes, as there are no studies comparing the different modalities of treatment for CS in pregnancy. However, evidence suggests that patients receiving treatment during pregnancy achieve better foetal outcomes than those who do not receive treatment. We aim to summarise in this review the major diagnostic and management difficulties during pregnancy.

KEY WORDS: Cushing syndrome - Cushing disease - Pregnancy - Foetal outcomes

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Publication History

Article first published online: February 13, 2018
Manuscript accepted: January 29, 2018
Manuscript received: January 13, 2018

Cite this article as

Caimari F, Corcoy R, Webb SM. Cushing's disease: major difficulties in diagnosis and management during pregnancy. Minerva Endocrinol 2018 Feb 13. DOI: 10.23736/S0391-1977.18.02803-1

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