Recent Updates on the Diagnosis and Management of Cushing's Syndrome PDF

Review Endocrinol Metab 2018;33:139-146 https://doi.org/10.3803/EnM.2018.33.2.139 Article pISSN 2093...

Review Endocrinol Metab 2018;33:139-146 https://doi.org/10.3803/EnM.2018.33.2.139 Article pISSN 2093-596X · eISSN 2093-5978 Recent Updates on the Diagnosis and Management of Cushing’s Syndrome Lynnette K. Nieman Diabetes, Endocrine and Obesity Branch, The National Institute of Diabetes and Digestive and Kidney Diseases (NIDDK), National Institutes of Health, Bethesda, MD, USA Cushing’s syndrome, a potentially lethal disorder characterized by endogenous hypercortisolism, may be difficult to recognize, espe- cially when it is mild and the presenting features are common in the general population. However, there is a need to identify the con- dition at an early stage, as it tends to progress, accruing additional morbidity and increasing mortality rates. Once a clinical suspicion is raised, screening tests involve timed measurement of urine, serum or salivary cortisol at baseline or after administration of dexa- methasone, 1 mg. Each test has caveats, so that the choice of tests must be individualized for each patient. Once the diagnosis is es- tablished, and the cause is determined, surgical resection of abnormal tumor/tissue is the optimal treatment. When this cannot be achieved, medical treatment (or bilateral adrenalectomy) must be used to normalize cortisol production. Recent updates in screening for and treating Cushing’s syndrome are reviewed here. Keywords: Cushing syndrome; Hydrocortisone; Adrenocorticotropic hormone INTRODUCTION However, many of these, for example weight gain and hyper- tension, are common in the general population. As a result, cli- Cushing’s syndrome denotes pathologic hypercortisolism as a nicians must use information about the tempo and duration of result of excessive adrenocorticotropic hormone (ACTH) pro- signs and symptoms, as well as biochemical testing, to establish duction, or autonomous adrenal production of cortisol. This po- the diagnosis of Cushing’s syndrome. tentially lethal disorder is associated with significant comorbidi- After establishing the diagnosis, its cause must be determined, ties, including hypertension, diabetes, coagulopathy, cardiovas- because the ideal treatment is surgical resection of the abnormal cular disease, infections, and fractures. As a result, even after tumor/tissue. If surgery is not possible or there is recurrent or cure of hypercortisolism, mortality rates may be increased. Be- metastatic disease, medical therapy (or bilateral adrenalectomy) cause of this it is important to make the diagnosis as early in the is chosen to normalize cortisol levels. Recent understanding disease course as possible, to prevent additional morbidity and related to screening for and treating Cushing’s syndrome will be residual disease. reviewed here. In general, clinical features suggest the syndrome (Table 1). Received: 11 April 2018, Revised: 23 April 2018, Accepted: 16 May 2018 Copyright © 2018 Korean Endocrine Society Corresponding author: Lynnette K. Nieman This is an Open Access article distributed under the terms of the Creative Com­ Diabetes, Endocrine and Obesity Branch, The National Institute of Diabetes and mons Attribution Non-Commercial License (http://creativecommons.org/ Digestive and Kidney Diseases (NIDDK), National Institutes of Health, Building licenses/by-nc/4.0/) which permits unrestricted non-commercial use, distribu­ 10, CRC, 1 East, Rm 1-3140, 10 Center Dr, MSC 1109, Bethesda, MD 20892- tion, and reproduction in any medium, provided the original work is properly 1109, USA cited. Tel: +1-301-496-8935, Fax: +1-301-402-0884, E-mail: [email protected] www.e-enm.org 139  Nieman LK Table 1. Signs and Symptoms of Cushing’s Syndrome SUSPECTING AND SCREENING FOR CUSHING’S SYNDROME Systemic Fatigue or hypomania (may alternate) When to suspect Cushing’s syndrome Growth retardation (in children) Increased weight from baseline Cushing’s syndrome should be considered when a patient pres- Insomnia ents with signs and symptoms of the syndrome (Table 1) that Vivid dreams cannot be explained in another way. Because many of the Skin, adipose, hair features may cluster under a single diagnosis, it is very impor- Abnormal adipose in dorsocervical, supraclavicular, temporal areas tant for subspecialists to look for features outside of their sub- Striae, especially if >1 cm width and purple ject matter expertise, to avoid missing the diagnosis. Some ex- Thin skin, especially at