From the Guidelines
Cushing syndrome is a serious medical condition characterized by excess cortisol levels, requiring prompt diagnosis and treatment to minimize morbidity, mortality, and improve quality of life. According to the most recent consensus guideline update on the diagnosis and management of Cushing's disease, published in The Lancet Diabetes and Endocrinology in 2021 1, it is essential to accurately diagnose and manage Cushing syndrome to optimize patient outcomes.
Key Characteristics of Cushing Syndrome
- It occurs due to prolonged exposure to high levels of cortisol, which can be caused by taking high doses of corticosteroid medications or when the body produces too much cortisol on its own.
- Common causes include the use of oral corticosteroid medications, tumors in the pituitary gland (Cushing's disease), adrenal gland tumors, or other tumors that produce adrenocorticotropic hormone (ACTH).
Symptoms and Complications
- Symptoms include weight gain, fatty deposits, stretch marks, thinning skin, muscle weakness, fatigue, high blood pressure, high blood sugar, and increased thirst and urination.
- If left untreated, Cushing syndrome can lead to severe complications such as diabetes, bone loss, and increased risk of infections, highlighting the importance of early diagnosis and treatment.
Treatment Approach
- Treatment depends on the underlying cause but may involve reducing or stopping corticosteroid medications under medical supervision, surgery to remove tumors, radiation therapy, or medications that control cortisol production, as recommended by the consensus guideline update 1.
- The goal of treatment is to reduce cortisol levels to normal, manage symptoms, and prevent long-term complications, thereby improving the patient's quality of life and reducing morbidity and mortality.
From the FDA Drug Label
The FDA drug label does not answer the question.
From the Research
Definition and Causes of Cushing Syndrome
- Cushing syndrome is defined as a prolonged increase in plasma cortisol levels that is not due to a physiological etiology 2
- The most frequent cause of Cushing syndrome is exogenous steroid use, but the estimated incidence of Cushing syndrome due to endogenous overproduction of cortisol ranges from 2 to 8 per million people annually 2
- Cushing disease, in which corticotropin excess is produced by a benign pituitary tumor, occurs in approximately 60% to 70% of patients with Cushing syndrome due to endogenous cortisol production 2
Clinical Presentation of Cushing Syndrome
- Cushing syndrome is associated with hyperglycemia, protein catabolism, immunosuppression, hypertension, weight gain, neurocognitive changes, and mood disorders 2
- Characteristic skin changes include facial plethora, easy bruising, and purple striae, while metabolic manifestations include hyperglycemia, hypertension, and excess fat deposition in the face, back of the neck, and visceral organs 2
Diagnosis and Evaluation of Cushing Syndrome
- Evaluation of patients with possible Cushing syndrome begins with ruling out exogenous steroid use 2
- Screening for elevated cortisol is performed with a 24-hour urinary free cortisol test or late-night salivary cortisol test or by evaluating whether cortisol is suppressed the morning after an evening dexamethasone dose 2
- Plasma corticotropin levels can help distinguish between adrenal causes of hypercortisolism and corticotropin-dependent forms of hypercortisolism 2
Management and Treatment of Cushing Syndrome
- Management of Cushing syndrome begins with surgery to remove the source of excess endogenous cortisol production, followed by medication that includes adrenal steroidogenesis inhibitors, pituitary-targeted drugs, or glucocorticoid receptor blockers 2
- Adrenally directed medical therapies, such as ketoconazole, metyrapone, osilodrostat, mitotane, and etomidate, can be used to inhibit adrenal steroidogenesis 3
- Steroidogenesis inhibitors, such as ketoconazole, metyrapone, levoketoconazole, and osilodrostat, can be used alone or in combination to control hypercortisolism 4