Cushing's syndrome medical therapy
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Medical therapy
The medications usually used for the treatment of Cushing's syndrome are:
- Pasireotide
- Cabergoline
- Ketoconazole
- Metyrapone
- Mitotane
- Mifepristone
Drug regimen
- Pasireotide 750–2400 μg per day subcutaneously injected
- Cabergoline Up to 7 mg per week orally
- Ketoconazole 400–1600 mg per day orally
- Metyrapone 0·5–4·5 g per day orally
- Mitotane 3–5 g per day orally
- Mifepristone 300–1200 mg per day orally
The main point to remember is that the vast majority of cases of Cushing's syndrome are caused by steroid medications (such causes are described as iatrogenic) so healthcare workers must always remember to check patients' medications first, as stopping or reducing the dose will usually resolve the problem.
If an adrenal adenoma is identified it may be removed by surgery. An ACTH-secreting corticotrophic pituitary adenoma should be removed after diagnosis. Regardless of the adenoma's location, most patients will require steroid replacement postoperatively at least in the interim as long-term suppression of pituitary ACTH and normal adrenal tissue does not recover immediately. Clearly, if both adrenals are removed, replacement with hydrocortisone or prednisolone is imperative.
In those patients not suitable for or unwilling to undergo surgery, several drugs have been found to inhibit cortisol synthesis (e.g. ketoconazole, metyrapone) but they are of limited efficacy.
Removal of the adrenals in the absence of a known tumor is occasionally performed to eliminate the production of excess cortisol. In some occasions, this removes negative feedback from a previously occult pituitary adenoma, which starts growing rapidly and produces extreme levels of ACTH, leading to hyperpigmentation. This clinical situation is known as Nelson's syndrome.[1]
References
- ↑ Nelson DH, Meakin JW, Thorn GW. ACTH-producing tumors following adrenalectomy for Cushing's syndrome. Ann Intern Med 1960;52:560–569. PMID 14426442