This protocol addresses patients with Ectopic ACTH syndrome who develop life-threatening complications of Cushing's syndrome, or who have severely elevated urine free cortisol (more than five times the upper limit of normal). Urgent intervention — within 24 to 72 hours — is indicated.
Management centres on urgent cortisol reduction using a combination of steroidogenesis inhibitors, with alternative options available for patients who cannot take oral medications. Each associated complication — including thromboembolic and infectious — requires targeted intervention alongside cortisol control. The complete regimen, agent selection, and sequencing are in the full protocol.
Reduction of urine free cortisol to near-normal within 24 to 48 hours of initiating treatment.
DOI: 10.1210/jc.2015-1818
We recommend urgent treatment (within 24–72 h) of hypercortisolism if life-threatening complications of CS such as infection, pulmonary thromboembolism, cardiovascular complications, and acute psychosis are present.
Many experienced clinicians suggest specific treatments for each condition (eg, anticoagulation prophylaxis and prophylaxis for Pneumocystis jiroveci with trimethoprim-sulfamethoxazole [or dapsone in patients with sulfa allergies]), especially for bedridden or low-mobility patients or those with UFC >5-fold normal.
The associated disorder(s) should be addressed as well (eg, anticoagulation, antibiotics).
In 11 critically ill patients, a high-dose regimen combining mitotane (3.0–5.0 g/d), metyrapone (3.0–4.5 g/d), and ketoconazole (400–1200 mg/d) decreased UFC to near normal levels within 24–48 hours with dramatic improvement in clinical condition and acceptable side effects.
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