Cushing's disease

Medical quality assurance by Dr. Albrecht Nonnenmacher, MD at November 2, 2016
StartCushing's disease

Cushing's disease is a disorder that occurs when the body has a high level of the hormone, cortisol. Cushing's disease is a type of Cushing's syndrome which specifically begins with a tumor. That tumor is benign and is usually located in the pituitary gland.

Contents

Definition and facts

It is a rare disease that only affects about 10 to 15 people for every million per year. It mostly affects people ages 20 to 50, and about 70 percent of those are women. Most patients have small tumors which can be difficult to diagnose. An endocrinologist will usually assist with the diagnosis. 

The adrenal glands produce cortisol which helps control blood pressure and keeps the cardiovascular system working properly. It also helps in dealing with stress and how the body converts fats, proteins and carbohydrates into energy it can use. 

Symptoms & Complaints

There are many symptoms to signal the onset of Cushing's disease. These include:

Women can also have extra growth of hair on their body (hirsutism) and irregular periods or no periods. Men may experience reduced [[libido|sex drive, infertility, or erectile dysfunction. When children acquire Cushing's syndrome, parents will notice glowed growth.  

Other symptoms that can occur as the disease progresses are severe fatigue, muscle weakness, psychological problems, high blood pressure and bones fracturing easily. Some may have cognitive difficulties, or problems with decision-making and thought-processing skills.

Causes

The cause of Cushing's disease is an excess amount of the hormone cortisol in the blood level which is usually caused by a tumor of the pituitary gland or hyperplasia (increased growth) of the pituitary gland. Pituitary gland tumors such as pituitary adenomas can trigger excessive release of adrenocorticotropic hormone, (ACTH) which is triggered by a signal from the hypothalamus. ACTH signals the adrenal glands to release cortisol.

A rare genetic disorder known as MEN 1 or multiple endocrine neoplasm 1 disorder can cause pituitary gland tumors to develop, which then causes Cushing's.

Diagnosis & Tests

Cushing's disease can often be difficult to diagnose and can be overlooked until it is in the later stages of the disease. Doctors will perform a thorough physical examination and ask about the patient's medical history as well as their families' medical histories and what symptoms they have now and have had in the past. They will investigate all symptoms from now and the recent past to determine if they are factors for the disease.

Medical professionals will check for tumors through medical imaging studies such as magnetic resonance imaging (MRI) scans and computed tomography (CT) scans, and will also conduct blood tests and clinical urine tests for elevated ACTH levels and cortisol levels. They may test the saliva for levels of cortisol.

Treatment & Therapy

The goals of treatment for Cushing's disease are to get hormone levels back to normal and keep them there, to keep this from becoming a chronic condition.

The first treatment doctors will try and pursue is surgery to remove any pituitary tumors. Radiotherapy may be necessary to treat patients whose tumors could not be completely removed.

Medications such as mitotane, metyrapone, and ketoconazole may be prescribed to reduce the levels of cortisol in the body.

Prevention & Prophylaxis

Cushing's disease is largely caused by tumors of the pituitary gland that are genetic or idiopathic in origin. As such, it cannot be prevented. Seeking prompt diagnosis and treatment may help mitigate long-term complications such as infertility, osteoporosis, and obesity.