Cushing’s syndrome or hypercortisolism is a condition caused by elevated levels of cortisol in the body. It is accompanied by various clearly visible changes in appearance and body dysfunction. Cushing’s syndrome must be treated because it progresses and becomes life-threatening without therapy.
What is Cushing’s syndrome?
Cushing’s syndrome is a condition in which several different symptoms are triggered by an increase in cortisol levels. Cortisol is a hormone produced in the adrenal cortex. A distinction is made between endogenous (originating in the body) and exogenous (caused externally) Cushing’s syndrome. In endogenous Cushing’s syndrome, the adrenal cortex produces more cortisol than the body needs due to abnormal changes. Exogenous Cushing’s syndrome is caused externally when glucocorticoids (cortisone) or ACTH are given to the body over a long period of time as part of a therapy. ACTH is a hormone that stimulates the adrenal cortex to produce more cortisol. If there is a permanent oversupply of cortisol in the body, the typical symptoms of Cushing’s syndrome occur, with changes in appearance and various functional disorders. Cushing’s syndrome is a rare disease, with only 3-4 cases occurring annually among 100,000 people.
Causes
The cause of Cushing’s syndrome is always an excess of the hormone cortisol. In exogenous Cushing’s syndrome, this is caused by the administration of drugs that are given over a long period of time as part of a therapy. These are cortisol-containing drugs that are taken, for example, in the case of chronic inflammations, after transplantations or in the case of autoimmune diseases. If the drugs are discontinued after successful treatment, the symptoms usually disappear. Endogenous Cushing’s syndrome is caused by an overproduction of the hormone cortisol in the body. There are various reasons for this increased release of cortisol. A common trigger for endogenous Cushing’s syndrome is a tumor on the pituitary gland. Some type of lung cancer and adrenal tumors can also be triggers.
Symptoms, complaints, and signs
The first symptom of Cushing’s syndrome is often an altered distribution of fat on the body: Fat is increasingly deposited on the face (“full moon face”), the neck thickens (“buffalo neck”), and abdominal girth increases. Arms and legs are only slightly muscled and appear very thin compared to the torso. Due to increased fat deposits, body weight increases, muscle mass and thus muscle strength decrease. Increased cortisol secretion can affect bone density, and bones become brittle and break more easily. This often results in bone and back pain. It is not uncommon for diabetes mellitus to develop as a result of hypercortisolism: signs include great thirst and increased urination. Headaches and elevated blood pressure are also common in Cushing’s syndrome. Externally, the disease is manifested in many cases by acne, wound healing disorders, the increased occurrence of bruises and a strong body hairiness in women. The skin becomes thinner, and red stripes can form on the abdomen, hips and in the armpit area, resembling stretch marks during pregnancy. In women, menstrual disorders develop, and menstruation may not occur at all. Men often suffer from potency disorders, children often show growth disorders with simultaneous obesity. In many cases, depression, anxiety attacks, severe mood swings, and increased susceptibility to infections accompany the disease.
Diagnosis and course
The symptoms of Cushing’s syndrome are very diverse. Often, a diagnosis is not made until years later, since the symptoms develop only gradually and are not immediately apparent. Typical symptoms at first are weight gain, high blood pressure, and symptoms similar to those of diabetes, that is, increased thirst and frequent bladder emptying. Over time, the body changes in appearance. The face becomes rounder and is usually reddened, fat is deposited in the neck and the so-called buffalo or bull neck develops. Muscle strength decreases and back pain occurs.Men often have potency problems, women do not menstruate and have increased body hair. Kidney stones, osteoporosis and heart failure may occur. Psychological changes are also possible. Some sufferers experience anxiety attacks, depression or mood swings. The diagnosis of Cushing’s syndrome can only be confirmed by various laboratory tests. As a rule, saliva, blood and urine are examined with special laboratory tests. Furthermore, imaging techniques are used such as sonography (ultrasound), computed tomography (tomograms) and scintigraphy (images with contrast medium). If Cushing’s syndrome is not treated, it can lead to a life-threatening condition in the long run.
When should you see a doctor?
Most often, the symptoms of Cushing’s syndrome occur as side effects during cortisone therapy. This is the exogenous form of the disease, which is brought on from the outside. It becomes alarming when the symptoms exceed the level announced by the treating physician. If it is an overdose, the doctor will slowly reduce the dose. The cause of the increased complaints could also be due to an additional drug that the patient is taking without the doctor’s knowledge. To avoid incompatibilities, the information on medications must be complete during the medical history. If a previously healthy person develops typical Cushing’s symptoms, a visit to the doctor is unavoidable. If Cushing’s is suspected, the family physician will refer the patient to a specialist in endocrinology. Using tests, imaging techniques and physical examinations, he or she will diagnose the trigger for the symptoms. If the cause is a tumor leading to increased cortisol production, the endocrinologist will advise surgery and then initiate appropriate therapy. Untreated Cushing’s syndrome can be life-threatening. Important body systems are imbalanced in this disease. Without therapy, there is a risk of stroke or heart attack. Therefore, a visit to the doctor should not be postponed. With timely treatment, the prognosis is positive in most cases.
Treatment and therapy
Treatment of Cushing’s syndrome depends on the cause. The goal is always to return elevated cortisol levels to normal so that the physical changes caused can regress. In the case of exogenous Cushing’s syndrome, the triggering drugs are discontinued step by step or, if they are still therapeutically necessary, at least reduced. In endogenous Cushing’s syndrome, the cause of the overproduction of cortisol must be eliminated. Often surgery is necessary to remove the tumor causing the overproduction, sometimes radiation is also used. If a tumor exists directly on the adrenal gland, removal of one or both adrenal glands may also be necessary. After such an operation, the patient must take hormones for life (hormone replacement). If surgery is not possible for certain reasons, drugs that inhibit the formation of cortisol can help. Sometimes these drugs are given in the preparatory phase before surgery. After treatment of Cushing’s syndrome, cortisol levels must be monitored regularly over a long period of time.
Outlook and prognosis
The course of Cushing’s syndrome depends on the cause present as well as the timing of the start of treatment. If the syndrome is triggered by the administration of medications containing high levels of cortisol, relief of symptoms occurs immediately upon discontinuation of the medication. After a few weeks, Cushing’s syndrome is considered cured because the excess cortisol has been removed from the body and excreted. This is a side effect of medication administered within a long-term therapy. If the disease arises from a carcinoma, recovery depends on the site of tumor formation and the timing of diagnosis and initiation of treatment. If the carcinoma is detected early, there is a good chance of recovery. If an existing tumor has already spread further in the organism, the prospects of recovery decrease. In the case of bronchial carcinoma, the prospects are very unfavorable. In contrast, renal carcinoma can lead to recovery if removed in time. In the case of a benign ulcer in the area of the pituitary gland, the prospects of recovery from Cushing’s syndrome are considered good.The ulcer severely limits the activity of the pituitary gland. If the tumor is detected in time, it can be treated and removed. As a result, the pituitary gland resumes its natural function and the patient experiences healing.
Prevention
One can only prevent exogenous Cushing’s syndrome. When cortisol-containing drugs are used in therapy, regular monitoring of cortisol levels can immediately detect an elevation and prompt action can be taken. Prevention of endogenous Cushing’s syndrome is not possible.
Follow-up
Some measures and options for aftercare are available to the affected person in hypercortisolism, although these usually depend very much on the exact cause of the disease, so that general predictions can be made in the process. However, the disease must be detected at a very early stage to avoid fatal complications or further worsening of symptoms. Therefore, in the case of hypercortisolism, the affected person should see a doctor at the first symptoms and signs and treat this disease. If hypercortisolism is caused by medications, these must be discontinued. However, medical monitoring should always take place. In case of interactions or if there is any uncertainty, a doctor should always be contacted first. Furthermore, in some cases surgical interventions are necessary to alleviate the symptoms of hypercortisolism. After such an operation, the affected person should in any case rest and take care of his body. Effort and other physical or stressful activities should be avoided. When taking hormones, care should be taken to ensure correct dosage and also regular intake. Whether there is a reduced life expectancy due to this disease, can not be universally predicted in the process.
This is what you can do yourself
The possibilities of a help to self-help is not possible with Cushing’s syndrome with a direct influence on the course of the disease. The focus of an everyday support for yourself should therefore be the mental and emotional well-being. Life with the disease should be adapted and optimized to the given possibilities. A healthy lifestyle, a balanced diet and a stable social environment are beneficial. The use of help from people in the close environment should take place free of inhibitions, without demanding too much from others. In the case of psychological problems, therapeutic support is helpful. This can be used to develop behavioral strategies that can be used above all to cope with challenging situations. Exchanging ideas with people who have the same diagnosis can also be beneficial. With mutual tips and advice on how to better deal with the disease, this can bring relief. A good and confident self-confidence is very helpful in everyday life, especially in contact with the public. In order to be prepared for the developments of the disease, there should be a comprehensive exchange of information between the doctor and the patient. Alternatively, missing knowledge can be acquired via studies or specialist literature. This helps to avoid surprises and to be prepared for the occurrence of unpleasant situations.
