Department

Neuroendocrinology

Document Type

Article

Abstract

CONTEXT: Primary bilateral macronodular adrenal hyperplasia (BMAH) is a rare form of adrenal Cushing syndrome conventionally treated with adrenalectomy. Medical treatment is often reserved for patients not eligible for surgery. However, to date there have been few studies about the efficacy of mifepristone for the treatment of BMAH associated with hypercortisolism.

OBJECTIVE: To describe a series of patients with hypercortisolism due to BMAH treated with mifepristone from multiple medical practices.

DESIGN: We retrospectively assessed four patients treated with mifepristone for hypercortisolism due to BMAH who had either failed unilateral adrenalectomy, declined surgery, or were poor surgical candidates.

RESULTS: Mifepristone induced clinical improvement and remission of the signs and symptoms of hypercortisolism in all described patients with BMAH. The median treatment duration at the time of efficacy response assessment was 5 months (range: 3 to 18 months). Improvement in cardiometabolic parameters was observed as early as 2 weeks after treatment was started. All patients achieved improvements in glycemic control and hypertension and had significant weight loss. The most common adverse event observed with mifepristone therapy was fatigue. Increases in TSH level occurred in two patients.

CONCLUSION: Mifepristone can be an effective medical alternative to surgery in patients with hypercortisolism due to BMAH.

Medical Subject Headings

Adrenal Hyperplasia, Congenital; Adult; Aged; Female; Hormone Antagonists; Humans; Magnetic Resonance Imaging; Male; Middle Aged; Mifepristone; Prognosis

Publication Date

12-1-2019

Publication Title

The Journal of clinical endocrinology and metabolism

ISSN

1945-7197

Volume

104

Issue

12

First Page

6279

Last Page

6290

PubMed ID

31112270

Digital Object Identifier (DOI)

10.1210/jc.2018-02638

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