Medical management of Cushing's disease: When and how?

Transsphenoidal surgery is the first-line treatment of Cushing's disease. However, medical treatment can be given in a high proportion of patients with this rare condition. This is especially the case in severe hypercortisolism for which medical treatment will be given for a short period of time to prepare the patient for surgery, or on a long-term basis after failed transsphenoidal surgery, or when waiting for the maximal beneficial effect of radiation techniques. These different situations all require that hypercortisolism be perfectly controlled. Severe hypercortisolism is frequently treated by a block and replace approach during which a substitutive treatment is given after the induction of adrenal insufficiency. Mild hypercortisolism, the most frequent situation, is usually treated with a titration approach with a progressive dose increase. This approach requires certainty of eucortisolism because patients may be treated for a prolonged period. This review details the main situations during which medical treatment should be considered, as well as the way in which to monitor them to avoid the long-term consequences of mild hypo- or hypercortisolism.