The most common cause for Cushing syndrome and its high levels of cortisol is regular, long-term use of oral steroids. The best treatment depends on the underlying cause but could involve gradually stopping steroid medications. If symptoms are caused by tumors, treatment options may include medications, surgery, or radiation therapy.

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Cushing syndrome, also called hypercortisolism, is identified by the body making too much of the hormone cortisol, which has many important roles such as regulating your body’s reactions to stress.

You can develop this syndrome through long-term use of certain medications or because of complications from cancerous tumors, and the treatments are specific to what may have led to the syndrome.

In this article, we take a deeper look at when a doctor or healthcare professional may recommend each of the many different therapies and what those treatment options involve.

Before examining the treatment options, here’s a quick refresher on Cushing syndrome.

This condition is characterized by abnormally high cortisol levels, which may be 4 times higher than the upper limit of what’s considered normal.

People with this syndrome may develop symptoms that include:

Causes of Cushing syndrome

The most common cause of Cushing syndrome is the long-term use of oral steroid medications.

It’s rare that Cushing syndrome is caused by tumors, with this 2019 study estimating the risk at 3.2 people per 1 million per year.

Difference between Cushing disease and syndrome

Cushing disease is a specific type of Cushing syndrome.

It occurs when your pituitary gland produces too much of the hormone adrenocorticotropic hormone (ACTH), which leads to the overproduction of cortisol.

Cushing disease is the most common cause of Cushing syndrome not related to medications.

The best treatment for Cushing syndrome depends on your underlying condition. A doctor may recommend:

  • reducing or stopping oral steroids
  • surgery to remove a tumor
  • radiation therapy
  • medications
  • specific treatment for cancers such as lung cancer

The best treatment for Cushing syndrome depends on the underlying cause.

Reducing corticosteroid use

Regular, long-term use of oral corticosteroids such as prednisone is the most common cause of Cushing syndrome. Corticosteroids are synthetic drugs that resemble the hormone cortisol. Tapering off steroids is usually the best treatment option when medications are causing your symptoms.

It’s generally recommended to wean off these drugs slowly because it can take months for the function of your adrenal glands to fully recover.

Surgery

A doctor may recommend surgery if your Cushing syndrome is caused by a tumor.

A procedure called transsphenoidal surgery is usually used to remove tumors on the pituitary gland. This surgery involves removing the tumor through your nose.

Pituitary tumors cause about 70% of cases of nonsteroid use Cushing syndrome in adults and 60 to 70% in children. Transsphenoidal surgery has a cure rate of about 80 to 90% if the tumor is located.

If you have a tumor on an adrenal gland, a doctor may recommend an adrenalectomy. This surgery involves removing an adrenal gland. It can often be done as a minimally invasive procedure using a long, thin tube called a laparoscope.

Radiation therapy

Radiation therapy may be recommended if transsphenoidal surgery fails. It may also be recommended if you aren’t a candidate for surgery.

According to the American Association of Neurological Surgeons, radiation therapy is generally administered over 6 weeks and has led to improvements in about 40 to 50% of adults and up to 85% of children.

A procedure called stereotactic radiosurgery is another potential treatment option. During this procedure, doctors use precisely focused radiation beams to destroy cancer. In a 2021 study, researchers found 13 out of 16 people treated with a form of this procedure called Gamma Knife radiosurgery had normalized hormone levels at a 2-year follow-up.

Medications

Medications that block the production of cortisol are another potential option to treat people not eligible for surgery or who don’t respond to surgery. These include:

  • ketoconazole
  • mitotane
  • metyrapone
  • mifepristone

If your ACTH levels drop below a normal range after pituitary surgery, a doctor might prescribe corticosteroids, such as hydrocortisone or prednisone, to compensate.

Signifor injection

Signifor injections contain the active ingredient pasireotide. They were FDA-approved in 2012 to treat Cushing disease when surgery isn’t a treatment option or isn’t effective. It’s usually injected into a muscle at the top of your thigh or abdominal area.

In a 2018 clinical trial, researchers found that once-monthly injections normalized cortisol levels in about 40% of people in a group of 150 people with Cushing disease.

Isturisa pills

Isturisa is the first FDA-approved medication that works by blocking the enzyme 11-beta-hydroxylase to prevent the production of cortisol. It’s approved to treat Cushing disease when surgery isn’t an option or effective.

In a clinical trial with 137 adults, 86% of people who received Isturisa kept cortisol levels within normal limits compared with 30% of people taking a placebo. Most people in the study either had undergone pituitary surgery or weren’t candidates for surgery.

Ectopic Cushing syndrome makes up about 5 to 20% of cases of Cushing syndrome.

It occurs when cancer cells create excess ACTH that leads to elevated cortisol levels. The most common cause is lung cancer, specifically a type called small-cell lung cancer (SCLC). It’s estimated that up to 12% of people with this cancer develop Cushing syndrome.

Treating ectopic Cushing syndrome largely involves removing the underlying cancer. In the case of SCLC, treatment usually includes some combination of:

Having diabetes or another health condition can complicate Cushing syndrome treatment.

With diabetes in particular, higher levels of cortisol can lead to blood sugar spikes — which make diabetes management more challenging and can negatively impact a person’s overall health. Some medications, such as Signifor injections, have the potential to further raise your blood sugar levels.

Those higher blood sugars can lead to someone being less likely to heal quickly, meaning they might be ineligible for surgery.

Diabetes can also develop as a side effect of Cushing syndrome, which then creates a vicious circle of making it more difficult to treat Cushing syndrome itself.

Getting your diabetes and glucose levels in range is a critical part of your treatment plan in managing Cushing syndrome. Treating diabetes in people with Cushing syndrome is essentially the same as treating it in people without Cushing syndrome.

The type 2 diabetes medication metformin is a first-line therapy for higher blood sugar levels (hyperglycemia) in people with Cushing disease.

Cushing syndrome symptoms usually resolve after proper treatment, but improvements might take months to over a year.

Eating a balanced diet and adopting overall healthy lifestyle habits may help you manage your symptoms while you’re waiting for your hormone levels to return to normal.

Corticosteroids can suppress adrenal gland function, and it can take as long as 18 months for your body to start producing a normal amount of cortisol again.

After surgery, corticotropin-releasing hormone levels usually return to normal within 12 months.

In a 2016 case study, researchers found improvements in the physical symptoms of Cushing syndrome and blood pressure in a 37-year-old woman with a tumor on one adrenal gland 6 months after she had her adrenal gland removed.

It can take years for cortisol levels to stabilize after radiation therapy.

Cushing syndrome is characterized by extremely high levels of cortisol many times over the upper limit of what’s normal. Medical treatment with a doctor’s guidance is needed to treat underlying causes such as tumors.

Many websites promote the use of herbal remedies for treating Cushing syndrome. However, these haven’t been found to be effective or safe.

Some herbal remedies can actually induce Cushing syndrome. For example, in a 2017 case study, researchers presented the case of a 5-year-old girl who developed Cushing syndrome after a herbalist prescribed an unregistered ointment for eczema with undisclosed dexamethasone.

Cushing syndrome is a disorder characterized by abnormally high levels of cortisol. The most common cause is regular, long-term use of oral steroids. The best treatment depends on the underlying cause.

If corticosteroid medications are causing your symptoms, a doctor may recommend slowly weaning off them. The best treatment option for tumors is generally surgery. Medications and radiation therapy may be options if you don’t respond to surgery or aren’t eligible.