ACTH-Independent Cushing Syndrome

Cushing syndrome from autonomous adrenal cortisol production.

Written by: Saygı Hospital Health Guide Editorial Board

Last updated: June 10, 2026

This content has been compiled by the Saygı Hospital Health Guide Editorial Board and is periodically reviewed by a specialist physician.

References (5)

This content is for informational purposes only and does not constitute medical advice. You can book an appointment at our Endokrinoloji department. Book Appointment →

What is ACTH-Independent Cushing Syndrome?

ACTH-independent Cushing syndrome is a form of Cushing syndrome resulting from autonomous cortisol production by the adrenal cortex, with suppressed ACTH levels. The principal causes are adrenocortical adenoma, adrenocortical carcinoma, ACTH-independent macronodular adrenal hyperplasia (AIMAH), and primary pigmented nodular adrenocortical disease (PPNAD).

The most common cause of the ACTH-independent form is an adrenal adenoma, which is usually unilateral. Carcinoma is rare but follows an aggressive course and often produces androgenic features as well.

Diagnosis is established with biochemical tests (24-hour urinary cortisol, late-night salivary cortisol, low-dose dexamethasone suppression test) and imaging (CT/MRI); a suppressed ACTH level is an important clue.

Symptoms

Moon facies and central obesity

Dorsocervical 'buffalo hump'

Purple striae and thin skin

Muscle weakness and easy bruising

Hypertension and diabetes

Psychiatric changes (depression, anxiety)

Osteoporosis and fractures

Risk Factors

Adrenal incidentaloma

Age (middle-aged and older)

Carney complex (PPNAD)

McCune-Albright syndrome (AIMAH)

Family history of adrenal tumors

Female sex (adenoma)

Rarely, ectopic adrenal tissue

When to See a Doctor?

If you experience any of the following symptoms, seek medical attention promptly:

Typical features suggesting Cushing syndrome
Resistant hypertension and new-onset diabetes together
When an adrenal incidentaloma is detected
Rapid weight gain and purple striae
Easy bruising and history of fractures

Treatment Methods

Unilateral adrenalectomy in unilateral adenoma (curative)

Radical surgery and mitotane in adrenocortical carcinoma

Bilateral adrenalectomy in AIMAH and PPNAD

Temporary glucocorticoid replacement after surgery

Medical therapy: ketoconazole, metyrapone, osilodrostat

Lifelong endocrinology follow-up

Which Department to Visit?

You can visit our Endokrinoloji department for these complaints. Our specialist physicians will create the most suitable treatment plan for you.

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Health Disclaimer: The information on this page is prepared for general informational purposes only. It does not replace medical diagnosis and treatment. Please consult your physician for your complaints. Saygı Hospital does not accept responsibility for actions taken based on the information on this page.