Medical Management of Cushing’s Disease

Chapter
First Online:
Part of the Endocrine Updates book series (ENDO, volume 31)

Abstract

Because established therapies for Cushing’s disease – surgery and radiation – are not consistently successful and have clear limitations with respect to efficacy and side effects, there is a need for an effective medical treatment. The most commonly used drugs act by inhibition of steroidogenesis in the adrenals, with the greatest experience available for ketoconazole and metyrapone. Although effective, access to these compounds is limited in many countries, long-term experience is scarce, and side effects are significant. When choosing between these agents, metyrapone may be preferable to ketoconazole in males, and vice versa in women, because of their association with hirsutism and hypogonadism, respectively. Recent proof-of-concept studies suggest a role for a pituitary-directed therapy, applying new multireceptor ligand somatostatin analogs such as pasireotide or second-generation dopamine agonists. However, larger multicenter studies are required to establish their long-term efficacy and safety. The glucocorticoid receptor antagonist mifepristone offers an alternative approach, but thus far experience is very limited, and dosing needs to be titrated with respect to clinical improvements rather than conventional hormonal assays, as there is no biochemical assay to monitor efficacy. Hopefully, currently ongoing studies of these different approaches will increase the possibilities for medical treatment of this fascinating, but sometimes dreadful, disease.

Keywords

Steroidogenesis Hirsutism Hypogonadism Adrenal-directed therapy Pituitary-directed therapy Multireceptor ligand somatostatin 
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© Springer Science+Business Media, LLC 2011

Authors and Affiliations

  1. 1.ENDOC Center for Endocrine TumorsHamburgGermany
  2. 2.University of Duisburg-EssenEssenGermany

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