Description
About the Case Discussion Topic: Cyclic Cushing Syndrome is a rare form of Cushing Syndrome characterized by intermittent episodes of cortisol excess followed by periods of normal cortisol levels. This cyclical pattern can make diagnosis particularly challenging, as hormone levels may appear normal during testing unless caught during an active phase. The condition may be caused by pituitary adenomas, ectopic ACTH secretion, or adrenal tumors. Symptoms often fluctuate with the cortisol cycles and may include weight gain, hypertension, fatigue, and mood changes. Diagnosis typically requires serial cortisol measurements over time to detect the episodic nature of hormone excess.
Summary Listen
- Cushing's Syndrome, a rare disease characterized by chronic high-barcortizolemia, affects 0.2 to 5 per million people. Cyclic Cushing's Syndrome (CCS), a subtype, presents challenges in diagnosis due to its intermittent nature, with periods of high and normal cortisol levels. It may represent 15-18% of Cushing's Syndrome patients.
- The etiology of CCS mirrors classic Cushing's, stemming from pituitary, adrenal, or ectopic causes. Clinical symptoms are also similar, including obesity, skin changes, hypertension, and mood disturbances, although some studies suggest more psychiatric symptoms in CCS patients, particularly among women.
- Diagnosis relies on lab tests, including the Dexamethasone suppression test, late-night salivary cortisol, and urinary free cortisol. These tests can be unreliable due to the fluctuating cortisol levels in CCS. Newer methods like scalp hair cortisol analysis and desmopressin stimulation test can help catch the episodic nature of CCS.
- The case definition for CCS generally requires two to three peaks of high cortisol levels separated by one to two troughs of normal levels, with trough duration ranging from hours to months. Female patients between 50-60 years old and more inclined to alcohol consumption are more likely to develop CCS.
- The treatment approach for CCS mirrors classic Cushing's, prioritizing surgery to address the underlying cause. Medical management includes steroid synthesis inhibitors and glucocorticoid replacement therapy in a "block and replace" strategy. Novel agents such as osilodrostat, are also used for controlling cortisol levels.
- Emerging biomarkers like hair cortisol and the desmopressin stimulation test are used to improve detection and recurrence prediction. Continuous glucose monitoring may also be used to detect periods of hyperglycemia associated with high cortisol levels. A current international trial focuses on determining the prevalence of CCS and the best monitoring timeframe for accurate diagnosis.
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About the Speakers
Dr. Ihab El Tayeb
Chairman & Founder of International Diabetes and Obesity Conference, Dubai, UAE
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