Overview
Cushing syndrome (CS) results from chronic exposure to excess cortisol, often due to a pituitary adenoma (Cushing disease) or ectopic ACTH secretion, leading to a wide array of metabolic, musculoskeletal, and psychological complications 126.Diagnosis
Clinical Features: Fatigue, weight gain, central obesity ("buffalo hump"), muscle weakness, hypertension, and skin changes 13.
Biochemical Tests: Elevated 24-hour urinary free cortisol, midnight salivary cortisol, or low-dose dexamethasone suppression test 4.
Imaging: MRI of the pituitary gland to identify pituitary adenomas; CT or MRI of chest/abdomen to detect ectopic sources 6.
Differentiating Causes: Differentiate between pituitary and ectopic ACTH sources through plasma ACTH levels and imaging studies 26.Management
Surgical Intervention: Transsphenoidal surgery for pituitary adenomas; resection or targeted therapy for ectopic sources 26.
Medical Therapy:
- Dexamethasone: For temporary control of hypercortisolism 4.
- Metyrapone: Inhibits cortisol synthesis 4.
- Aldosterone Antagonists: For management of hypertension 1.
Radiation Therapy: Post-surgical for pituitary adenomas if remission is not achieved 2.Special Populations
Pregnancy: Management requires careful consideration of teratogenic risks and fetal well-being; close monitoring and multidisciplinary care essential 1.
Elderly: Increased risk of comorbidities; tailored management focusing on quality of life and minimizing adverse effects 1.Key Recommendations
Early Diagnosis and Treatment: Critical to mitigate long-term morbidity and mortality; systematic screening in high-risk populations may improve outcomes (Evidence: Moderate 4).
Comprehensive Follow-Up: Essential to monitor persistence of comorbidities despite remission; regular assessments for cardiovascular, metabolic, and musculoskeletal health (Evidence: Moderate 1).
Tailored Management Plans: Consider individual patient factors such as age and comorbidities to optimize treatment efficacy and safety (Evidence: Expert opinion 1).References
1 Puglisi S, Perini AME, Botto C, Oliva F, Terzolo M. Long-Term Consequences of Cushing Syndrome: A Systematic Literature Review. The Journal of clinical endocrinology and metabolism 2024. link
2 Limumpornpetch P, Morgan AW, Tiganescu A, Baxter PD, Nyawira Nyaga V, Pujades-Rodriguez M et al.. The Effect of Endogenous Cushing Syndrome on All-cause and Cause-specific Mortality. The Journal of clinical endocrinology and metabolism 2022. link
3 Hoenig LJ. The Buffalo Hump of Cushing Syndrome. Clinics in dermatology 2022. link
4 Lam-Chung CE, Cuevas-Ramos D. The promising role of risk scoring system for Cushing syndrome: Time to reconsider current screening recommendations. Frontiers in endocrinology 2022. link
5 Runderawala H, Shah S, Manked A. Adrenal Oncocytoma - A Rare Functional Tumor Presenting as Cushing Syndrome. The Journal of the Association of Physicians of India 2017. link
6 Sathyakumar S, Paul TV, Asha HS, Gnanamuthu BR, Paul MJ, Abraham DT et al.. ECTOPIC CUSHING SYNDROME: A 10-YEAR EXPERIENCE FROM A TERTIARY CARE CENTER IN SOUTHERN INDIA. Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists 2017. link
7 Harris C. Clinical Perspective: What Do Addison and Cushing Tell Us About Glucocorticoid Action?. Advances in experimental medicine and biology 2015. link