This protocol addresses severe Cushing's disease — severe hypercortisolism — with potentially life-threatening metabolic, psychiatric, infectious, or cardiovascular/thromboembolic complications. In these patients, rapid normalization of cortisol is the most important goal.
A combination of adrenal steroidogenesis inhibitors (including ketoconazole plus metyrapone) was used to control severe hypercortisolism, but normalization of cortisol levels was not achieved. This protocol describes the recommended next step after that failure.
When optimized medical therapy, including combination steroidogenesis inhibition, has not controlled hypercortisolism, a definitive surgical intervention is considered to achieve rapid and durable cortisol reduction.
For patients with severe disease, rapid normalization of cortisol is the most important goal.
Patients with severe CD who have potentially life-threatening metabolic, psychiatric, infectious, or cardiovascular/thromboembolic complications also may benefit from preoperative medical therapy in select cases.
However, if hypercortisolism is very severe and not responsive to optimized medical therapy, including combinations, BLA should be considered to avoid worsening outcomes.
However, BLA may be warranted earlier in patients with severe hypercortisolism in whom a rapid, definitive effect on cortisol is needed to avoid prolonged systemic effects of uncontrolled disease.
DOI: 10.1016/S2213-8587(21)00235-7
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