Cushing's syndrome

Cushing's syndrome versus Cushing's disease

It's important to understand the difference between these two terms. Cushing's syndrome is the general name for any condition where there is too much cortisol in the body, regardless of the cause. Cushing's disease is a specific type of Cushing's syndrome. It occurs when a small growth in the pituitary gland (a pea-sized gland in your brain) causes the body to make too much cortisol25.

Cushing's disease is the most common cause of Cushing's syndrome that comes from inside the body. It accounts for about 70% of cases in adults and 60% to 70% of cases in children and teenagers25.

• Adrenal glands
• Pituitary gland
• Hypothalamus
• Kidneys

Medication-related causes

The most common cause of Cushing's syndrome is taking high doses of steroid medicines, such as prednisone or hydrocortisone, for a long time. These medications, called glucocorticoids, are used to treat conditions like asthma, rheumatoid arthritis, lupus, and other autoimmune diseases. More than 10 million Americans take glucocorticoids each year, though not all develop Cushing's syndrome236.

If you are taking steroid medicine and develop symptoms of Cushing's syndrome, it's very important not to stop taking your medication suddenly without talking to your doctor first. Stopping these medicines abruptly can make you very unwell4.

Tumor-related causes

When Cushing's syndrome comes from inside your body, it's usually caused by a tumor. Most of these tumors are small, noncancerous growths. They can occur in several places67:

• A tumor in the pituitary gland that makes too much ACTH (a hormone that tells your adrenal glands to produce cortisol). This is the most common cause, accounting for about 70% of cases
• A tumor in one or both adrenal glands that produces too much cortisol directly
• A tumor somewhere else in the body, such as the lungs or pancreas, that produces ACTH. This is called ectopic Cushing's syndrome
• Overgrowth of the adrenal glands

Physical changes

One of the most noticeable signs of Cushing's syndrome is changes in how your body looks. Weight gain is common, but it happens in a specific pattern. Fat tends to build up around the face (making it look round, sometimes called "moon face"), the upper back and shoulders (creating a hump called a "buffalo hump"), and the chest and stomach area. Meanwhile, your arms and legs may become thinner as you lose muscle12.

Skin changes are also common. Your skin may become thin and fragile, bruising easily from minor bumps. You might develop stretch marks that are pink or purple in color, especially on your stomach, hips, thighs, breasts, and under your arms. Wounds and cuts may heal slowly. Some people develop acne or notice more facial and body hair growth (a condition called hirsutism)16.

Physical symptoms

Many people with Cushing's syndrome experience muscle weakness, particularly in the upper arms and legs. This weakness can make everyday activities more difficult. Feeling tired or exhausted is also very common. Some people develop bone pain or find that their bones break more easily18.

Hormonal and reproductive symptoms

Women may notice that their menstrual periods become irregular or stop completely. They may also experience excessive hair growth on the face and body. Men may have problems with erections and notice a decreased interest in sex. Both men and women can experience a lower sex drive16.

Emotional and mental symptoms

Cushing's syndrome doesn't just affect your body; it can also impact your emotional and mental well-being. The hormone imbalance can contribute to mood swings, making you feel irritable, anxious, or depressed more often than usual. You may experience difficulty focusing, problems with memory, or have trouble controlling your emotions. Some people describe feeling as though they are in a "mind fog"29.

Symptoms in children

In children, the main signs of Cushing's syndrome are obesity and being shorter than expected for their age. Children with this condition often grow slowly16.

Initial evaluation

Your doctor will start by reviewing all your medicines, including pills, injections, creams, and inhalers, to see if you're taking any steroid medications that could cause the condition. If you are taking such medicines, you may not need other tests10.

During a physical examination, your doctor will look for signs of Cushing's syndrome, such as a round face, a hump on the back of your neck, thin skin with bruising, and stretch marks110.

Testing cortisol levels

Several tests can be done to measure the amount of cortisol in your body. It's not uncommon to need multiple tests, which may take weeks to months for conclusive results11.

A 24-hour urine test requires you to collect all your urine over a full day. The laboratory will measure the cortisol and other hormones in this sample. Blood tests can also measure cortisol and ACTH levels at different times of the day110.

A saliva test is particularly useful because cortisol levels normally drop in the evening in people without Cushing's syndrome. By collecting a small sample of saliva at night, doctors can see if your cortisol levels are staying too high when they should be dropping110.

Your doctor may also use a dexamethasone suppression test. You will be given a medication called dexamethasone at night, and then your blood will be drawn the next morning. This test measures whether your cortisol levels drop normally after taking this medication11.

Finding the cause

Once high cortisol is confirmed, your doctor will need to find out what's causing it. Imaging tests such as CT scans or MRI scans can take pictures of your pituitary gland and adrenal glands to look for tumors. Additional blood tests may be done to measure different hormones and help identify where the problem is located10.

Surgery

When Cushing's syndrome is caused by a tumor, surgery to remove the tumor is usually the first-line treatment. This is the preferred option because it can cure the condition while keeping your body's natural hormone system intact1312.

If the tumor is in the pituitary gland, surgeons typically use a minimally invasive approach, going through the nose to reach the gland. This type of surgery is successful in bringing about disease remission in around 78% of patients. However, in about 13% of patients, the condition may come back within 10 years after surgery, meaning that nearly one third of patients may need additional treatment in the long term514.

If the tumor is in an adrenal gland, that gland will need to be removed. In some cases, both adrenal glands may need to be removed. After this surgery, you will need to take medicines to replace the hormones that your adrenal glands normally make for the rest of your life6.

Radiation therapy

If surgery is not successful or cannot be done, radiation therapy may be used to shrink or destroy a tumor in the pituitary gland. Radiation is effective in controlling cortisol excess in a large percentage of patients, but it may take months or even years to work fully. It can also cause hypopituitarism, a condition where the pituitary gland doesn't produce enough hormones, requiring long-term hormone replacement614.

Medical therapy

Several medications can be used to control cortisol levels or block its effects on the body. Medical therapy has become increasingly important in treating Cushing's syndrome, especially with the development of new drugs in recent years1415.

Medications may be used in several situations: before surgery to help improve your health and reduce risks; after surgery if it was not completely successful; while waiting for radiation therapy to take effect; or, in selected cases, as the primary treatment when surgery is not an option14.

There are different types of medications. Some drugs work directly on the adrenal glands to reduce cortisol production. Ketoconazole is one of the most commonly used drugs in this category because it acts quickly. Other medications block the action of cortisol at its receptors throughout the body. Mifepristone is particularly effective for controlling symptoms related to high blood sugar and has been approved for treating Cushing's syndrome when it's associated with diabetes. A newer drug called pasireotide works on the pituitary gland and has been approved for treating Cushing's disease when surgery has failed or is not an option1415.

All medications require careful monitoring by your doctor, along with clear instructions for you and your caregivers15.

Adjusting steroid medication

If your Cushing's syndrome is caused by taking steroid medicine, your doctor may try to gradually reduce your dose or switch you to a different medication. This must be done carefully and slowly under medical supervision66.

The recovery process

Recovery from Cushing's syndrome takes time. Many patients feel worse immediately after successful surgery than they did before the operation. This is normal and expected. You may experience fatigue, weakness (sometimes described as feeling like a "wet noodle"), muscle and joint pains, and dry skin16.

It's important to be patient with yourself. You didn't develop symptoms overnight, and you won't recover quickly either. For most people, it takes 6 to 12 months to recover from Cushing's syndrome, though some aspects may take longer. The rate of recovery varies greatly from person to person and depends on how long you had the condition, how severe your symptoms were, and your age. Younger patients tend to recover more quickly than older patients16.

One encouraging point is that emotional symptoms such as irritability, mood swings, and depression often improve before the physical problems do. However, memory problems may take longer to resolve16.

Managing emotional and mental health

The emotional challenges of living with Cushing's syndrome can feel overwhelming. Many people experience feelings of anger, frustration, sadness, loneliness, and self-loathing. These feelings are common among people with chronic diseases9.

It's important to talk to your healthcare provider about your feelings. Your emotional health is just as important as your physical health. If you have depression or anxiety, these can be treated separately from Cushing's syndrome. Consider seeking help from a mental health professional if needed9.

Try to surround yourself with family and friends who are supportive and understanding of your situation. Connecting with others who have Cushing's syndrome through support groups can help you feel less alone9.

Dealing with cognitive problems

High cortisol levels can make it hard to focus and remember things. Some people describe this as a "mind fog." Training your brain to focus for longer periods can help improve your memory9.

Try these strategies: practice daily meditation to improve focus; use self-talk or cues to help remember important information; repeat important details to help your brain store them; use sticky notes as reminders; organize your day with checklists or planners; and engage all your senses when trying to remember something. Don't be hard on yourself if you're having memory or attention problems—it's the disease, not you9.

Lifestyle adjustments

Making healthy lifestyle choices can support your recovery and overall well-being. Eat a healthy diet that is high in protein and calcium to help prevent muscle and bone loss. Limit salt in your diet, especially if you have high blood pressure. Regular exercise is important—even gentle activities like walking can help. Talk with your doctor about how much exercise is safe for you1718.

A physical therapist can help develop an exercise program tailored to your needs. Simple exercises can be done at home without special equipment to help with rehabilitation, symptom relief, and general wellness18.

Get regular eye exams to check for glaucoma and cataracts, which can develop as complications of Cushing's syndrome. See your doctor regularly to monitor and treat any complications such as diabetes, high blood pressure, or bone problems17.

Work and daily life

If your symptoms make it difficult to work, you may be able to file for disability benefits. Talk to your healthcare provider about your options and what documentation you might need. Let your employer, family, and friends know what you're going through so they can better understand and support you9.

Long-term follow-up

Follow-up care is a key part of your treatment and safety. Make sure to attend all your appointments, even after your cortisol levels have returned to normal. The condition can sometimes return, so ongoing monitoring is important. Call your doctor if you're having problems or if your symptoms don't improve as expected17.

With proper treatment and support, many people with Cushing's syndrome can lead active, fulfilling lives. The journey may be difficult, but there is hope and help available. You are not alone19.