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Cushing's syndrome due to primary bilateral macronodular adrenal hyperplasia

Author
André Lacroix, MD
Section Editor
Lynnette K Nieman, MD
Deputy Editor
Kathryn A Martin, MD

INTRODUCTION

Among the causes of Cushing's syndrome are three rare types of nodular adrenocortical diseases that are usually bilateral:

Corticotropin (ACTH)-dependent bilateral macronodular adrenal hyperplasia, secondary to long-term adrenal stimulation in patients with Cushing's disease or ectopic ACTH syndrome. However, the adrenals rarely reach the massive size that can be seen with primary bilateral macronodular adrenal hyperplasia, and plasma ACTH may still be detectable.

ACTH-independent micronodular adrenal hyperplasia and its most frequent variant, primary pigmented nodular adrenocortical disease (PPNAD), which may be sporadic or familial (as part of the Carney complex).

Primary bilateral macronodular adrenal hyperplasia (BMAH), a disorder previously referred to as ACTH-independent bilateral macronodular adrenal hyperplasia (AIMAH).

BMAH will be reviewed here. Other causes of Cushing's syndrome and PPNAD are discussed separately. (See "Causes and pathophysiology of Cushing's syndrome" and "Cushing's syndrome due to primary pigmented nodular adrenocortical disease".)

                              
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Literature review current through: Nov 2017. | This topic last updated: Apr 21, 2017.
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