Hormone Problems in Patients: Exploring the Link between Adrenal Insufficiency and Cushing’s Disease

by time news

2023-06-02 14:59:41

What do a patient who collapses in stressful situations and a patient with buffalo hump have in common? Both have a hormone problem! Read here what’s behind it.

Without Cortisol Things aren’t going well in the body: The hormone that is essential for survival is known to play an important role in the regulation of the circulatory system and immune system. In stressful situations such as an accident, a serious infection or a sports competition, the hormone keeps the circulatory system stable by increasing the metabolism and blood pressure. Furthermore, it has an anti-inflammatory effect. A lot of functions – a lot of problems when the hormone is not available to the extent that it should.

When the adrenal glands lack motivation

A press conference by the German Society for Endocrinology (DGE) highlighted such exceptional cases. Prof. Stephan Petersenn from the ENDOC Practice for Endocrinology and Andrology, Hamburg, dealt with the one extreme at the event, the adrenal insufficiency. Adrenal insufficiency leads to cortisol deficiency, which in turn leads to low blood pressure, low blood sugar and excessive inflammatory reactions. In the worst case, it can lead to a life-threatening shock.

There are many different causes of adrenal fatigue, as Petersenn explains. The most common are autoimmune diseases of the adrenal glands, such as Morbus Addison, which directly damage the adrenal glands, thereby reducing cortisol production. from one secondary adrenal insufficiency is the speech when the function of pituitary is disturbed, so that the release of the adrenal stimulating hormones CRH and ACTH disturbed – by autoimmune diseases or tumours.

However, not only endogenous factors, but also exogenous influences can put a stop to cortisol production: under long-term high-dose glucocorticoid therapy, it is possible that the pituitary and adrenal glands “forget” to produce sufficient cortisol themselves. This is also known as iatrogenic adrenal insufficiency. An abrupt discontinuation of such a therapy can also lead to a deficiency of the hormone; the medication must therefore always be gradually tapered off to give the body time to resume its own production.

Diagnosis: only for experts

Unfortunately, the diagnosis of adrenal insufficiency is often made late, when a full-fledged adrenal crisis has already occurred. Because the symptoms of the disease initially develop insidiously and are anything but specific: loss of performance, fatigue, arthralgia and myalgia, psychological abnormalities such as psychoses, depression, and memory disorders also occur in other, more widespread diseases. Nausea, vomiting, or abdominal pain are less common hypoglycemia.

Even apart from the anamnesis, the diagnosis is not without. An early morning cortisol measurement can give a first meaningful indication – however, the cortisol level is subject to many different influencing factors, which makes measurement and interpretation more difficult. “It is therefore important that these tests are carried out by competent hands,” says Petersenn. “A certain level of expertise is required.” Provocation tests like a insulin hypoglycemia test, a Metopiron-Test or a ACTH-Test can help to confirm the suspected diagnosis of adrenal insufficiency.

A little more cortisol would be nice…

The good news, however, is that the lack of cortisol in everyday life can be easily compensated for by substitution therapy with hydrocortisone or synthetic ones Glucocorticoids can be replaced. The dosage should imitate the daily rhythm. A central part of the therapy is the thorough training of patients and relatives and the filling out of an emergency card, which the patient should always carry with them. Because stressful situations continue to pose a challenge in therapy. In the case of an acute illness or during operations, for example, the dose should be adjusted upwards.

The training of patients in self-injection of cortisone for emergencies is also particularly important. Because even if the majority of patients present themselves to the hospital in good time at the beginning of a suspected adrenal crisis, they often do not receive timely emergency medication. In the case of such an emergency injection, the following applies to both patients and doctors: do not be squeamish and “rather give a little more than be afraid of side effects”. These are actually only to be expected in the case of a chronic overdose, reassures Petersenn. “A possible one-time overdose is less serious than the risk of dying from shock due to undersupply.”

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… but not permanently!

Prefer a little more – the motto only applies to one-off doses. Over the long term, too much cortisol also causes significant damage throughout the body. With long-term high-dose glucocorticoid therapies, for example in rheumatic diseases, caution is advised and blood pressure, blood sugar and bone density should be checked regularly. But like a deficiency, an excess of cortisol can also arise due to endogenous processes. Diseases of the adrenal gland and the pituitary gland as well as endocrine tumors can not only reduce cortisol production, but also pathologically increase it.

A particularly serious clinical picture is in this respect Morbus Cushing. It is a rare hormonal disease caused by a pituitary adenoma. The tumor leads to increased release of ACTH and as a result to excessive production of cortisol. The result of this hormone overload is increased storage of fatty tissue in the neck and trunk area, while arms and legs lose muscle mass. Blood vessels and skin also react to the hormone and become more sensitive and thinner with the disease; this is shown, for example, by a tendency to hematomas or acne.

“Too much cortisol in the body leads to a variety of symptoms, almost every body tissue reacts to cortisol,” explains Apl.-Prof. Ilonka Kreitschmann-Andermahr, Senior Physician for Neurology and Special Pain Therapy at the Neurosurgical Clinic of the University Medicine Essen. These symptoms continue to include high blood pressure, osteoporosisand Typ-2-Diabetes. “We also have many cortisol receptors in the brain: it can lead to depression and other mental illnesses such as psychosis and mania.”

Germany, that’s better

The incidence of Cushing’s disease is around 1-3 new cases per 1,000,000 inhabitants per year. Unfortunately, as with adrenal insufficiency and many other rare diseases, the same applies here: It is often discovered too late. In Cushing’s disease, the average time to correct diagnosis is 3-4 years, and this window has not improved over the past 20 years. “Germany is not particularly quick to recognize Cushing’s disease and such rare diseases,” warns Kreitschmann-Andermahr. A worldwide meta-analysis was even able to show that patients in Germany are diagnosed significantly later than in comparison countries such as the USA, Italy and Great Britain. “Of the studies where you could measure these ranges, Germany was the worst,” summarizes the expert. “For comparison: USA 35 months, Germany 56.”

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delay with consequences

This delay is very tragic for the patients. Because in itself, Cushing’s disease is easily treatable with appropriate expertise and interdisciplinary cooperation. Neurosurgical or drug-based procedures or radiation therapy enable hormone levels to be stabilized, symptoms to be alleviated and serious secondary diseases to be prevented. Timely action can also normalize the life expectancy of patients – provided the disease is detected early. Otherwise the chronic sequelae such as depression, osteoporosis and diabetes persist or are slow to resolve. This has a lasting negative effect on the patient’s quality of life: “Every year that it is recognized too late creates suffering,” says Kreitschmann-Andermahr.

Where does this delay in diagnosis come from? This is not only because the disease is not so well known as a rare disease. Kreitschmann-Andermahr points out that the symptoms of the disease develop gradually and insidiously. Patients therefore often wait a long time (one to two years) before they see a doctor and then see different specialists, to whom they only ever present part of the symptoms – to the dermatologist with the acne, to the cardiologist with the blood pressure. “So it can happen that each specialist is only shown part of the larger clinical picture […] and no one puts the big picture, the various symptoms, together and says, ‘This could be Cushing’s disease.’”

Even if this explains the delay, a comparison with other countries shows that things can go faster. It is therefore important that more attention is paid to the clinical picture so that doctors, regardless of their specialty, are more likely to think of the diagnosis of Cushing’s disease when they have the appropriate symptoms.

Image source: Houcine Fish, Unsplash

#Fatigue #myalgia #cortisol

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