From time to time at Westchester Health, patients come to us having been diagnosed with Cushing’s disease, a rare endocrine disorder caused by abnormally excessive amounts of the hormone cortisol. It can also be caused by excessive growth of the pituitary gland, known as hyperplasia. Cushing’s disease mostly affects women, but men and even children can also develop it.
Although Cushing’s disease is not widespread—only 40-70 people out of 1 million have it— we as endocrinologists thought it might be helpful to share information about this disorder so that patients who are exhibiting symptoms can have a better idea of what treatment they should pursue in order to be as healthy and active as possible.
What causes Cushing’s disease?
The most common cause of Cushing’s disease is adenoma, a usually-benign tumor on the pituitary gland that causes this gland to produce too much cortisol, a hormone that is normally released during stressful situations. Cortisol controls the body’s use of carbohydrates, fats and proteins, and reduces the immune system’s response to swelling (inflammation). People with high blood cortisol levels often experience excess bodily fat and insomnia.
What is cortisol and what does it do?
Cortisol’s role in the body is to maintain homeostasis, reports University Health News. Along with the hormone adrenaline, cortisol increases when the body experiences high stress—such as during heavy exercise or in a dangerous situation.
Adrenaline kick a person into high gear to deal with the stress, while cortisol regulates blood sugar, prompting an immune response. When the stress is no longer present, adrenaline and cortisol levels should normalize. However, if that doesn’t happen and cortisol levels remain high, symptoms of Cushing’s disease can begin to appear.
Women are at higher risk
According to the Hormone Health Network, Cushing’s disease occurs more commonly in women, particularly those between the ages of 20 and 50. Other factors that increase a person’s risk of Cushing’s disease include:
- type 2 diabetes
- poorly controlled blood sugar levels
- high blood pressure
Cushing’s disease symptoms
Rather than just one or two, there is a wide range of symptoms associated with Cushing’s disease, including:
- Behavioral changes and/or irritability
- Bone loss
- Cognitive decline
- Diabetes and glucose intolerance
- Easy bruising (thin skin)
- Erectile dysfunction
- Excessive hair growth (face, neck, chest, abdomen, thighs)
- Excessive thirst and urination
- Fatty deposits (belly, base of the neck, hump at the shoulders, face)
- Frequent infections
- Large belly with thin arms and legs
- Low libido
- Menstrual problems (amenorrhea)
- Mood disorders
- Muscle weakness and wasting, especially in the legs
- Purple streaks on the abdomen
- Red cheeks
- Round face
- Weight gain
Having any these symptoms, however, does not mean you have Cushing’s disease. When the symptoms are caused by high cortisol from stress or medications, it’s called Cushing’s syndrome. It is only a disease if the elevated cortisol is caused by a pituitary gland or adrenal gland tumor. In either case, uncontrolled high cortisol levels are very damaging to your body’s systems.
Treatment and surgery
Treatment of Cushing’s disease is multi-dimensional and depends on the underlying cause. Many of the symptoms can be lessened or even reversed, but treatment must be administered carefully to minimize side effects and permanent hormone deficiency.
Without treatment, Cushing’s disease can lead to a shortened lifespan and reduced quality of life. High levels of cortisol brought on by the disease can cause other problems, including kidney stones, spinal compression fractures, infections, bone loss, muscle weakness and high blood pressure.
An individualized treatment plan needs to be created for each patient but usually includes:
While prescription drugs can reduce cortisol levels, when given alone they are not as effective as when part of long-term treatment, according to University Health News.
Drugs commonly used to treat Cushing’s disease are meant to:
- block the production of steroids derived from cholesterol (cortisol is produced by the adrenals from cholesterol)
- control excessive product of cortisol
- block the effect of cortisol in the tissues
- decrease the product of ACTH
2. Surgically removing the tumor
The only universally reliable treatment for Cushing’s disease is surgical removal of the tumor causing the abnormal release of cortisol. However, if the tumor is not completely removed, there is a chance (15%) that it will return. Radiation is often used following the surgery.
3. Surgically removing the adrenal glands
If removing the tumor is not successful, surgically removing the adrenal glands if often the next step. Following this, patients often need glucocorticoid therapy to offset the loss of cortisol.
Read our blogs on the subject
We’ve written several informative blogs focusing on medical conditions in the field of endocrinology, which you can read here.
Helpful websites we recommend
- Hormone Health Network
- Genetics Home Reference
- American Association of Neurological Surgeons
- National Organization for Rare Disorders
Concerned about Cushing’s disease? Please come see us.
If you have Cushing’s disease, or are worried that you’re at risk of developing it and would like guidance about treatment, please call (914) 232-1919 to make an appointment with one of our Westchester Health endocrinologists. We’ll examine you, evaluate your symptoms, possibly perform some tests, and together with you, decide on the best course of action going forward to improve your health and quality of life. Whenever, wherever you need us, we’re here for you.
By Mindy Sotsky, MD, FACE, a logist with Westchester Health, member of Westchester Health Physician Partners