Bilateral adrenal hyperplasia (BAH) and congenital adrenal hyperplasia (CAH) sound similar, but they differ in important ways. Both involve the adrenal glands, but they have different causes, symptoms, ages of onset, and treatment approaches.

Knowing the difference can help you manage your diagnosis, understand your treatment options, and have better conversations with your endocrinologist (hormone specialist).

What Are Adrenal Glands?

Your adrenal glands are small, triangle-shaped glands that sit on top of your kidneys. Each kidney has one adrenal gland. The glands are part of the endocrine system, which helps control your hormone levels.

🗳️ Which form of adrenal hyperplasia have you or your loved one been diagnosed with?

Bilateral adrenal hyperplasia (BAH)

Congenital adrenal hyperplasia (CAH)

A different form

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Adrenal glands produce hormones that help regulate metabolism, blood pressure, stress responses, and sexual development. They also make key hormones such as:

• Androgens — Sex hormones that affect normal development
• Cortisol — A “stress hormone” that controls blood pressure, blood sugar, and energy levels
• Aldosterone — A hormone that regulates blood pressure and salt levels

How Does Adrenal Hyperplasia Affect Your Adrenal Glands?

Adrenal hyperplasia refers to enlargement or overgrowth of adrenal tissue. Although adrenal enlargement can affect hormone production, it doesn’t always do so. Some types are hormonally active, while others may not cause significant hormone changes.

Adrenal hyperplasia is sometimes described using related terms like:

• Suprarenal hyperplasia — Another name for adrenal hyperplasia, as suprarenal means above the kidneys
• Adrenocortical hyperplasia — Enlargement of the adrenal cortex, the outer layer of the adrenal gland that produces cortisol, aldosterone, and androgens
• Nodular adrenal hyperplasia (also called nodular adrenocortical hyperplasia) — Hyperplasia involving one or more nodules within the adrenal glands

Although both BAH and CAH involve adrenal hyperplasia (enlarged adrenal tissue), they’re different conditions with different causes and treatments.

What Causes Bilateral and Congenital Adrenal Hyperplasia?

Although genetics can contribute to both BAH and CAH, these conditions are very different issues with the adrenal gland.

For example, CAH is an inherited genetic condition, while BAH is a descriptive term that includes several disorders that aren’t always caused by inherited genes.

How Does BAH Develop?

BAH can occur on its own or as part of a genetic syndrome. People with certain variations are more likely to have BAH.

Some forms of BAH, particularly PPNAD, are associated with variants in the PRKAR1A gene and may occur as part of the Carney complex.

In contrast, ARMC5 is one of the major genes linked to PBMAH. Studies show that ARMC5 variants occur in nearly 25 percent of PBMAH cases and are even more common in familial disease.

How Does CAH Develop?

In most cases, CAH develops from genetic variations in the instructions for making an enzyme called 21-hydroxylase.

Without enough 21-hydroxylase, the adrenal glands can’t make normal amounts of hormones, including cortisol and sometimes aldosterone. As a result, the adrenal glands work hard to make cortisol (although they can’t in CAH), produce excess androgens, and become large or hyperplastic.

CAH is inherited in an autosomal recessive pattern, meaning a person must inherit a nonworking copy of the gene from each of their parents.

How Do the Conditions’ Symptoms Differ?

Although both BAH and CAH affect the adrenal glands, their symptoms differ. These differences in symptoms are largely related to the adrenal hormones affected by each condition.

BAH Symptoms

Depending on which subtype you have, BAH may cause no noticeable changes or cause symptoms ranging from mild hormone imbalances to Cushing’s syndrome (a condition of excess cortisol). Symptoms can also vary significantly between individuals.

When an excess in cortisol does occur, symptoms may include:

• Hypertension (high blood pressure)
• A full, rounded face
• Unusually fast weight gain in the chest, belly area, and back of the neck
• Diabetes
• Excess hair on the chest, neck, breasts, face, and thighs
• Easy bruising and wounds that heal slowly

When BAH Symptoms Develop

BAH symptoms tend to develop later in life compared with those of CAH. For example, research shows that the median age at PPNAD diagnosis is 25, so half of people with PPNAD are diagnosed before 25, and half are diagnosed after.

Most people develop PPNAD in their 20s and 30s, but some children have it by age 3. On the other hand, PBMAH takes years to develop, and most people diagnosed are between ages 40 and 60.

CAH Symptoms

Unlike BAH, CAH is a congenital condition, meaning it’s present at birth and leads to a lack of cortisol. Symptoms may appear in infancy or shortly after birth, depending on the specific form of CAH and severity.

Some common CAH symptoms include:

• Differences in external genital development in infants
• Early puberty
• Acne
• Excess facial hair or body hair
• Irregular menstrual periods

The different forms of CAH, like salt-wasting, simple-virilizing, or nonclassical CAH, cause different symptoms like dehydration, vomiting, weight loss, early puberty, irregular periods, acne, and male-pattern baldness.

Classic CAH is often identified through newborn screening in countries that perform the screening, but if you know that CAH runs in your family, you may also choose to have early genetic testing done on family members and the individuals considering having a child.

The nonclassical form is usually milder and may be diagnosed in childhood, adolescence, or adulthood, depending on severity.

How Do Doctors Treat Bilateral and Congenital Adrenal Hyperplasia?

Treatment options vary depending on the cause of adrenal hyperplasia and whether the condition is causing symptoms or hormone abnormalities.

BAH Treatment Options

Some cases of BAH can be treated with adrenalectomy (surgery to remove one or both adrenal glands), especially when hormone excess causes symptoms or complications. Depending on the subtype and severity, other treatment options may include monitoring or medications.

CAH Treatment Options

CAH treatment focuses on managing hormone imbalances and preventing complications. Infants with the salt-wasting form need salt supplements to maintain healthy sodium levels.

Other medications help replace hormones the body can’t make on its own. Fludrocortisone, a synthetic mineralocorticoid, is used to replace low aldosterone levels. Cortisol is replaced with glucocorticoids (steroids) such as:

• Hydrocortisone (for growing children in particular)
• Prednisone
• Dexamethasone

High androgen levels may be managed with additional drugs including oral contraceptive pills and spironolactone. Crinecerfont (Crenessity) is a medication approved by the U.S. Food and Drug Administration (FDA) that may help lower the doses of steroids needed to control CAH symptoms.

Your endocrinologist will work with you to find the right treatment plan based on your individual needs.