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Glucocorticoid-Remediable Aldosteronism (GRA / FH-I)

Autosomal-dominant secondary hypertension from a chimeric CYP11B1/CYP11B2 gene placing aldosterone under ACTH control.

Written by: Saygı Hospital Health Guide Editorial Board
Last updated:

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

References (5)

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What is Glucocorticoid-Remediable Aldosteronism (GRA / FH-I)?

Glucocorticoid-remediable aldosteronism (GRA), also called familial hyperaldosteronism type I (FH-I), is a rare autosomal-dominant cause of secondary hypertension.

An unequal crossing-over creates a chimeric CYP11B1/CYP11B2 gene fusion that places aldosterone synthase under control of the ACTH-responsive 11-beta-hydroxylase promoter; aldosterone secretion is therefore driven by ACTH and suppressible by exogenous glucocorticoid.

Patients present with early-onset hypertension, variable hypokalemia, family history of stroke or aneurysm, and elevated 18-hydroxy-cortisol and 18-oxo-cortisol. Diagnosis is genetic; treatment uses low-dose dexamethasone or mineralocorticoid antagonists.

Symptoms

Early-onset hypertension (often before age 20)
Hypokalemia (variable, sometimes absent)
Headache, fatigue from hypertension
Muscle weakness with low potassium
Family history of intracranial aneurysms
Family history of hemorrhagic stroke
Resistant hypertension despite multiple drugs

Risk Factors

Family history of GRA or unexplained early hypertension
Affected first-degree relative
Chimeric CYP11B1/CYP11B2 gene mutation
Suppressed renin with high aldosterone-renin ratio
History of intracranial aneurysm in family
Hemorrhagic stroke at young age in family

When to See a Doctor?

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

  • Early-onset or resistant hypertension
  • Hypertension with hypokalemia
  • Strong family history of hypertension or stroke
  • Suppressed renin with elevated aldosterone-renin ratio
  • Genetic counseling for affected family
  • Pregnancy planning with GRA
  • Brain imaging for aneurysm screening

Treatment Methods

01
Low-dose dexamethasone (0.125-0.25 mg/d) or prednisone
02
Mineralocorticoid receptor antagonist (spironolactone, eplerenone)
03
Add-on antihypertensives (ACEi/ARB, calcium-channel blockers)
04
Genetic confirmation and family screening
05
Brain MRA for aneurysm screening (recommended)
06
Close blood-pressure and potassium monitoring
07
Lifestyle: low-sodium diet, exercise

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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