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Cushing’s disease: definition, symptoms, therapy

by Josephine Andrews
Published: Last Updated on 172 views

In Cushing’s disease , the body produces too much cortisol. One of the more common causes is a tumor in the pituitary gland. Women are affected more often than men. Complaints include obesity, loss of strength and increased susceptibility to infections. If the cause, such as a tumor, can be eliminated, the chances of recovery are good. Read all about the causes, symptoms and treatment of Cushing’s disease here.

ICD codes for this disease:

ICD codes are internationally valid codes for medical diagnoses. They can be found, for example, in doctor’s letters or on certificates of incapacity for work.

E24

quick overview

  • Symptoms: Changed fat distribution, trunk obesity, “moon face”, but relatively thin limbs, muscle weakness, bone loss, increased susceptibility to infections, in women: impure skin, signs of masculinization (eg strong facial hair)
  • Course of the disease and prognosis: Dependent on the cause, treatability and duration of the disease; often successful treatment possible, risk of secondary diseases such as diabetes mellitus, high blood pressure, cardiovascular diseases
  • Causes and risk factors: Overproduction of the hormone cortisol, eg due to tumors of the pituitary gland , tumors of the adrenal cortex, taking medication
  • Examinations and diagnosis: Various laboratory examinations, if necessary imaging procedures (MRI), ultrasound examination
  • Treatment: Depending on the cause, removal of the causative tumor by surgery, radiation, medication, rarely removal of the adrenal glands
  • Prevention: No targeted prevention, regular check-ups when taking glucocorticoids, no abusive use of steroids

What is Cushing’s disease?

Cushing’s disease was named after its describer, neurosurgeon Harvey Cushing. Patients suffering from Cushing’s disease have excessive amounts of cortisol in their body (hypercortisolism). Cortisol is a hormone produced in the adrenal cortex. It is considered a stress hormone and mobilizes energy reserves in stressful situations.

In order for cortisol to be formed in the adrenal cortex, it must be stimulated by another hormone: the adrenocorticotropic hormone (ACTH or corticotropin). ACTH is produced in the pituitary gland (pituitary gland). In Cushing’s disease, too much ACTH often circulates in the bloodstream, resulting in so-called ACTH-dependent hypercortisolism.

In addition to Cushing’s disease, there are other forms of hypercortisolism, such as Cushing’s syndrome, which are not caused by ACTH from the pituitary gland.

If Cushing’s disease develops spontaneously in the body, it is one of the so-called endogenous forms of hypercortisolism (endogenous = from within). This means that the body itself produces too much ACTH and therefore cortisol. In contrast, exogenous Cushing’s syndrome (caused from outside) develops when people take glucocorticoids or ACTH for a long time.

Endogenous hypercortisolism, in contrast to exogenous ones, is very rare. Experts estimate that around two to three out of a million people fall ill with it every year. Especially women between the ages of 30 and 40 are affected. About three quarters of them are thought to have a tumor in the pituitary gland (pituitary gland) that produces ACTH.

What are the symptoms of Cushing’s disease?

The following symptoms are typical of Cushing’s disease:

  • Redistribution of fat deposits: Fat is stored primarily on the torso (“main obesity”) and on the face. Therefore, patients have a so-called “full moon face” and a “bull neck”, but comparatively thin arms and legs.
  • Loss of strength: Muscle mass decreases (myopathy) and bones become brittle (osteoporosis).
  • Susceptibility to infection: Because the body’s defense system is partially suppressed by cortisol, pathogens multiply more easily. Wound healing is disturbed.
  • high blood pressure
  • Elevated blood sugar levels
  • Streaky, reddish discoloration of the skin (stretch marks, striae rubrae), especially on the upper arms and thighs and on the flanks
  • Thin, parchment-like skin that sometimes develops sores (ulcers).

Additionally, women with Cushing’s disease experience symptoms such as the following, which are due to an excess of male hormones :

  • cycle disorders
  • Change in hair type: women grow more hair on their face, chest and abdomen (hirsutism)
  • Masculinization (virilization): Women get a deeper voice, male body proportions or their clitoris grows.

In addition, some patients with Cushing’s disease develop psychological symptoms, such as depression. Growth arrest is more common in children with Cushing’s disease.

What is the life expectancy with Cushing’s disease?

Cushing’s disease does not have to limit life expectancy. The prognosis depends, among other things, on the cause and how well it can be treated. It also plays a role how long the disease has existed.

Due to the many different effects of cortisol on the body, there are sometimes various complications of Cushing’s disease. These include fractures, heart attacks and strokes.

If the reason is a tumor of the pituitary gland, surgery can cure Cushing’s disease in about 80 percent of cases. The ACTH level in the blood returns to normal shortly after the operation. If all other therapy options do not help and both adrenal glands are removed, patients must replace cortisol and mineral corticoids with medication for life.

What causes Cushing’s disease?

The main cause of Cushing’s disease is a microadenoma of the pituitary gland (pituitary gland) in 80 percent of cases. A microadenoma is a small tumor that is mostly benign. In a healthy body there are regulatory circuits that control the amount of hormones produced. A microadenoma is not subject to this control loop. Therefore, the amount of hormones in the body exceeds the required amounts.

If Cushing’s disease is present, increased corticotropin (ACTH) is produced. ACTH stimulates the adrenal cortex to produce cortisol. This is why it is also called ACTH-dependent hypercortisolism. ACTH also leads to the production of male hormones (androgens) in the adrenal cortex. This is why women with Cushing’s disease have symptoms of “masculinization.”

In addition to a microadenoma, there are other causes of Cushing’s disease.

In some cases there is a dysfunction of the hypothalamus . Corticoliberin (CRH) is produced in this brain area. This hormone stimulates the production of ACTH in the pituitary gland. Excessive amounts of corticoliberin from the hypothalamus induces increased production of ACTH in the pituitary gland, which ultimately leads to overproduction of cortisol in the adrenal cortex.

How is Cushing’s disease diagnosed?

If Cushing’s disease is suspected, your family doctor will refer you to a specialist in endocrinology. This is a specialist in metabolic and hormonal disorders. First, he will ask you in detail about your medical history (anamnesis). Among other things, he asks the following questions:

  • Have you gained weight?
  • Have your body proportions changed?
  • Do you have muscle or bone pain?
  • Do you get colds more often?

Your doctor will then examine you physically. He also pays attention to your skin color and possible skin changes. He will tap your spine and ask if you feel any pain. Your doctor will ask you to rise from a squat without arm support to test your muscle strength. He’ll also draw your blood.

Cushing’s disease: laboratory studies

Your blood will be tested in the laboratory for various values ​​that speak for Cushing’s disease. These include the amount of cortisol in your blood, the blood sugar level, the concentration of cholesterol, the number of immune cells and the concentration of electrolytes (especially the blood salts sodium and potassium ).

In Cushing’s disease, the amount of cortisol in the blood is elevated and doesn’t show different levels throughout the day as it normally would. The blood sugar level is increased because the liver produces more sugar (glucose). The cholesterol level is elevated. Various blood cells are increased ( leukocytes , thrombocytes and erythrocytes), while others are reduced (eosinophilic granulocytes and lymphocytes).

Cushing’s disease: specific tests

Furthermore, a so-called dexamethasone inhibition test is carried out. The patient is given dexamethasone (a glucocorticoid like cortisol) in the evening before bedtime. The next morning, the body’s own cortisol level in the blood should have fallen. In this way, the doctor proves that there is no hypercortisolism.

If cortisol levels are elevated the next morning, tests are done to confirm hypercortisolism. This includes, among other things, the determination of cortisol in the urine over a period of 24 hours. In Cushing’s disease, the value is increased.

In order to differentiate between the different forms of hypercortisolism, the amount of ACTH in the blood is now determined. When it is high, ACTH-dependent hypercortisolism is present, as is the case with Cushing’s disease.

Cushing’s disease: diagnostic imaging

The radiologist performs a magnetic resonance imaging (MRI) of the head. Tumors of the anterior pituitary can be seen on the MRI image. This is not always successful because the tumors are sometimes very small.

The adrenal gland can be visualized in an ultrasound examination (sonography) or computed tomography (CT). It is enlarged in Cushing’s disease.

Cushing’s disease: Other diseases with similar symptoms

Your doctor needs to differentiate Cushing’s disease from other diseases and triggers that cause similar symptoms and findings. These include, among other things:

  • Taking hormonal contraceptives (” pill “)
  • Taking steroids such as cortisone or sex hormones (without a doctor’s prescription)
  • Metabolic syndrome (disease consisting of obesity (obesity), high blood pressure and increased blood lipid levels)
  • Tumors of the adrenal cortex
  • diabetes mellitus
  • osteoporosis (bone loss)

How is Cushing’s disease treated?

If there is a microadenoma in the pituitary gland as the cause of Cushing’s disease, this is surgically removed. Neurosurgeons gain access to the pituitary gland through the nose or through the sphenoid bone (a bone at the base of the skull). After an operation, cortisol must be artificially administered for a short period of time.

If surgery is not an option, for example because of serious illnesses that make surgery impossible, or because no causative tumor was found, there are other treatment options. This includes taking medication. These work via different mechanisms. Possible active ingredients include, for example, pasireotide, ketoconazole or metyrapone.

Radiation to the pituitary is also a way to treat Cushing’s disease. This is how you destroy the microadenoma. It is rarely necessary to surgically remove both adrenal glands (adrenalectomy). This option is not a causal therapy and is rarely chosen when other therapy options have failed.

Patients then have to artificially replace cortisol and mineral corticoids, which are also produced in the adrenal cortex, with drugs for the rest of their lives.

Can Cushing’s disease be prevented?

Because most causes of Cushing’s disease, such as pituitary tumors, cannot be prevented, there is no single action that can prevent the disease.

However, for people who have to take glucocorticoids due to another underlying disease, it is advisable to pay attention to certain symptoms (eg weight gain, changed fat distribution). In the event of any abnormalities, it is advisable to speak to the doctor. As a rule, however, doctors prescribe glucocorticoids in a dosage that does not trigger Cushing’s symptoms.

In general, you should not take glucocorticoids or steroids (eg abused to build muscle) on your own without a medical reason or without a doctor’s prescription.

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