Bilateral adrenal hyperplasia (BAH) and congenital adrenal hyperplasia (CAH) sound similar, but they differ in important ways. Each condition causes a specific set of symptoms and tends to appear at different ages.

This article explains how BAH and CAH differ in causes, symptoms, and treatments. To learn more about BAH and CAH, talk with your endocrinologist (hormone specialist).

What Is Adrenal Hyperplasia?

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. According to Cleveland Clinic, the adrenal glands help:

• Regulate metabolism and energy
• Control blood pressure
• Manage the body’s response to stress
• Influence sexual development through androgen hormones

The adrenal glands are responsible for making key hormones, including:

• 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

The term “hyperplasia” means that tissue grows larger than it normally would. The cells in the adrenal glands divide more, making the glands bigger. Enlarged adrenal glands throw off your hormone balance.

What Are Bilateral Adrenal Hyperplasia and Congenital Adrenal Hyperplasia?

Most cases of adrenal hyperplasia are bilateral, meaning both glands are affected.

Research usually focuses on two forms of BAH, depending on how they affect the adrenal glands. Macronodular BAH leads to large growths or nodules on the adrenal glands. The micronodular form causes smaller nodules.

There are several types of BAH, including:

• Primary bilateral macronodular adrenal hyperplasia (PBMAH)
• Isolated micronodular adrenal disease (iMAD)
• Primary pigmented micronodular adrenal hyperplasia (PPNAD)

Both CAH and BAH involve adrenal hyperplasia but have different causes, symptoms, and treatment approaches.

What Causes Bilateral and Congenital Adrenal Hyperplasia?

Both BAH and CAH develop from genetic variations. The specific variation determines which condition develops.

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.

The most common variation linked to BAH affects a protein called PKA, which helps control how quickly cells grow and divide. The PRKAR1A gene provides instructions for making a piece of this PKA enzyme. Some variations cause cells to destroy this piece before it can be used. Without enough normal PKA proteins, adrenal gland cells grow out of control.

Researchers estimate that 26 percent to 60 percent of adrenal hyperplasias are caused by variations in the instructions for PKA proteins. Studies show that PRKAR1A gene variations lead to a condition called Carney complex. Tumors or growths on the adrenal glands are one sign of Carney complex. This can lead to a type of BAH known as PPNAD.

PBMAH and iMAD are less common types of BAH. Some cases result from variations that affect PKA proteins. Others are linked to variations in genes that help control when cells grow and divide.

How Does CAH Develop?

In most cases, CAH develops from genetic variations in the instructions for making an enzyme called 21-hydroxylase. Researchers describe CAH as an autosomal recessive disorder, meaning a person must inherit two nonworking copies of the gene — one from each parent — to have the condition.

A 21-hydroxylase deficiency disrupts cortisol production by the adrenal glands. In CAH, the adrenal glands make less cortisol and produce more androgens instead. This hormone imbalance is a key feature of CAH.

The classic type of CAH involves two main forms — salt-wasting (more severe) and non-salt-wasting (also called simple virilizing).

How Do the Conditions’ Symptoms Differ?

Although both BAH and CAH have genetic causes, their symptoms differ. People with BAH usually develop Cushing’s syndrome, a condition caused by too much cortisol. CAH can cause atypical sexual development due to high androgen levels. Other, more severe symptoms occur with salt-wasting CAH.

People with CAH can also develop Cushing’s syndrome if their glucocorticoid (steroid) dose remains too high for too long.

BAH Symptoms

Your specific BAH symptoms depend on what type of disease you have. Symptoms of Cushing’s syndrome (sometimes called hypercortisolism) 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

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. This means that 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.

PBMAH takes years to develop. Most people diagnosed with PBMAH are aged 40 to 60.

CAH Symptoms

CAH is a congenital condition, meaning it’s present at birth. Symptoms may appear in infancy or shortly after birth. BAH usually develops in adults.

CAH symptoms depend on the type you have. People with CAH have abnormally high androgen levels, which affect sexual development. According to Cleveland Clinic, CAH can lead to:

• Changes in genitalia in infants, such as an enlarged penis in males and atypical genitalia in females
• Signs of early puberty in younger children, like acne and facial or body hair growth
• Infertility or difficulty having children
• Abnormal menstrual cycles (periods)

Classic CAH is typically diagnosed at birth. Hospitals do newborn screenings for certain genetic conditions, including CAH. If you know that CAH runs in your family, you may also choose to have early genetic testing.

Nonclassic CAH is a milder form of CAH. Because symptoms are often subtle, nonclassic CAH may not be diagnosed until adolescence or adulthood. Signs of the nonclassic form include:

• Early puberty, such as acne and growth spurts
• Irregular or absent menstrual periods
• Male-pattern baldness
• Too much body or facial hair in females

Salt-wasting classic CAH is a severe form that results from very low aldosterone levels. Without enough of this hormone, you may experience:

• Low blood pressure
• Low blood sugar
• Low sodium levels
• Irregular heart rhythms
• Dehydration or loss of too much fluid
• Weight loss

• Vomiting

How Do Doctors Treat Bilateral and Congenital Adrenal Hyperplasia?

Treatment options vary depending on whether you have BAH or CAH.

Most BAH cases can be treated with surgery. Your endocrinologist will likely recommend removing one or both adrenal glands. Surgery is also used to help treat Cushing’s syndrome.

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

Other medications help replace hormones the body can’t make on its own. Synthetic mineralocorticoids (such as fludrocortisone) are used to replace low aldosterone levels. Cortisol is replaced with glucocorticoids such as:

• Dexamethasone
• Hydrocortisone
• Prednisone

High androgen levels may be managed with antiandrogens. These drugs include spironolactone and crinecerfont (Crenessity), a newer medication that may help lower steroid doses needed to control CAH symptoms.

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

Find Your Team

On CAHteam, people share their experiences with congenital adrenal hyperplasia, get advice, and find support from others who understand.

Are you or a loved one living with bilateral adrenal hyperplasia or congenital adrenal hyperplasia? Do you have other questions about the differences? Share your thoughts in the comments below.