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Living with Familial hyperaldosteronism type III

A rare inherited form of high aldosterone with severe high blood pressure that starts early, with enlarged adrenal glands.

Understanding Familial hyperaldosteronism type III

Familial hyperaldosteronism type III is a rare inherited condition with early-onset severe high blood pressure and excess aldosterone.

It does not respond to glucocorticoid (steroid) treatment. People may have very low potassium. It can appear at a variety of ages.

Signs & symptoms

Symptoms vary from person to person. GARD lists these among the most common:

  • High blood pressure (hypertension)
  • Enlarged adrenal glands
  • Abnormal renin levels
  • Headache
  • Muscle weakness
  • Extreme thirst

Not everyone has every symptom. GARD's list comes from the Human Phenotype Ontology.

Diagnosis

  1. Talk with your doctor

    Share the symptoms you've noticed, when they started and your family health history. Notes and photos can help.

  2. See a specialist

    Specialists who may be involved include genetic specialists, hormone specialists and kidney specialists.

  3. Consider genetic testing

    Changes in KCNJ5 are linked to this condition. Genetic testing and counseling can help confirm a diagnosis and explain what it means for your family.

  4. Connect with expert centers

    Primary Aldosteronism Foundation may keep lists of experts for this condition.

Care & treatment

Care team
Genetic specialists (geneticists); Hormone specialists (endocrinologists); Kidney specialists (nephrologists)
Expert care
Expert lists: Primary Aldosteronism Foundation
Treatment
GARD doesn't list specific treatments for this condition. Your care team will tailor care to your symptoms.
Research
Clinical trials and observational studies may be options. See the research section below.

General information, not medical advice. Your care team makes the decisions.

Everyday life

Living with a rare condition is easier with support.

  • Connect with Primary Aldosteronism Foundation and other patient organizations
  • Keep notes on symptoms, changes and questions for appointments
  • Ask about patient registries that help research
  • Look into financial aid and travel resources for care visits
Bring these questions to your next doctor visit
Can genetic testing for the KCNJ5 gene confirm this diagnosis?
Which treatments can control severe high blood pressure?
How should my heart and kidneys be monitored?

Quick facts

Symptoms usually starta variety of ages
InheritanceAutosomal Dominant
GenesKCNJ5

Changes in the KCNJ5 gene cause this condition. It is autosomal dominant, which means a change in one copy of the gene can cause it.

GeneticKidneyEndocrine

Also known as: familial hyperaldosteronism type 3; fh iii; fh-iii; fh3

Patient organizations

Source: NIH GARD · Familial hyperaldosteronism type IIIGARD ID 12362 · GARD page updated September 2026 · Plain-language summary by Know Rare; editorial review pending. General information, not medical advice.

You are more than your diagnosis

Perspectives from people navigating life with Familial hyperaldosteronism type III.

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