Diseases

Churg-Strauss Syndrome – Causes, Symptoms & Treatment

Churg-Strauss-Syndrom

Churg-Strauss syndrome is an inflammatory disease of the small blood vessels and belongs to the group of rheumatic diseases. Today it is also referred to in medicine as eosinophilic granulomatosis with polyangiitis ( EGPA ). The original naming goes back to the two American pathologists Jacob Churg and Lotte Strauss.

What is Churg-Strauss Syndrome?

In this disease, the arteries and veins at the end of the bloodstream are particularly affected and show permanent foci of inflammation. The Churg-Strauss syndrome is a primary vasculitis (vascular inflammation), there is no other underlying physical disease that causes this vascular inflammation.

The consequence of the constant inflammatory activity in the vessels is that the organs behind the diseased vessels are no longer supplied with sufficient blood and thus also with sufficient oxygen . Damage mainly occurs to the heart and lungs, but also to other internal organs, as well as to the skin and nerves.

causes

Churg-Strauss syndrome occurs primarily in patients who already suffer from asthma or another allergy . In addition to the asthmatic symptoms, granulomas develop, i.e. nodular inflammatory cells accumulate in the small vessels of the lungs and skin. These arise when the body’s own immune system turns against protein components of the blood vessel cells, fends them off and destroys them with the help of leukocytes .

This process is called an autoimmune reaction . The immune system turns against the body’s own components and destroys them. In the vessel itself, the body’s autoimmune reaction causes an inflammatory swelling of the vessel wall, which can completely block the vessel.

As a result, the adjacent organs can no longer be supplied with sufficient oxygen and nutrients. This is particularly dangerous in the heart arteries, as blocking them can lead to a heart attack and thus death.

Symptoms, Ailments & Signs

The symptoms of Churg-Strauss syndrome are rather non-specific at the beginning. First, allergic asthma occurs in most patients, associated with allergic rhinitis . In the further course it comes to pneumonia with fever . If the heart is affected by the foci of inflammation in the surrounding vessels, this manifests itself in cardiac insufficiency, which can lead to a heart attack if the inflammatory vessel is completely blocked.

Since the disease also damages individual nerve cells in many patients, numbness and paralysis can occur in the affected area. If the area around the stomach and intestines is affected, abdominal pain and diarrhea can occur. In the area of ​​the skin, the disease manifests itself in the form of ulcers and poorly healing, open wounds .

Diagnose

If other symptoms such as cardiac insufficiency, pneumonia, recurring inflammation of the paranasal sinuses , sores and bleeding on the skin occur in asthmatics, the doctor will arrange for further examinations. Tissue samples are taken from the affected skin areas or organs and examined.

Furthermore, an increased proportion of eosinophilic granulocytes, a subgroup of white blood cells, can be detected in the blood. In addition, certain antibodies directed against their own body structures can be detected in almost half of the patients suffering from Churg-Strauss syndrome.

In general, the laboratory findings of the blood test show increased inflammation values. With the help of X -rays , computed tomography and magnetic resonance imaging , foci of inflammation can be found in the heart, lungs or paranasal sinuses.

complications

Patients with Churg-Strauss symptoms suffer from constant inflammatory activity in the medium-sized and small arteries and veins, which destroys the affected vessels. The result is a reduced supply of blood to the organs located behind the damaged areas. Serious complications arise when the heart and lungs are affected.

Damage to kidneys, nerves, intestines and skin is also possible. The vascular inflammation is not based on other diseases that cause additional complications. However, patients with allergies, especially allergic asthma, belong to the risk group.

Taking certain medications can also lead to the complications of this vascular disease. Churg-Strauss syndrome is characterized by the accumulation of nodular inflammatory sites called granulomas. This results in an autoimmune disease, with which the body’s own defense system is directed against individual parts of the body.

The foci of inflammation multiply and cause vascular occlusion. In addition to allergic asthma, patients suffer from a runny nose, difficulty breathing and chest pain. Mild pneumonia with fever may occur. Diarrhea, weight loss and abdominal pain are associated symptoms of abdominal organ disease.

If individual nerves are damaged, there is a feeling of numbness in the supply area, which leads to symptoms of paralysis. If the heart is involved, the worst-case scenario is inflammation of the heart muscle, heart failure or a heart attack. The treatment is medicinal.

The survival rate of patients with Churg-Strauss syndrome depends on prompt successful therapy and is 60 percent, with cardiac involvement being crucial.

When should you go to the doctor?

Unfortunately, the Churg-Strauss syndrome does not show any particularly characteristic symptoms, so that early diagnosis and treatment of this disease is not possible in many cases. A doctor should be consulted if the affected person suffers from frequent colds and possibly also from asthma and other breathing problems. Fever and inflammation in the lungs and ears can also indicate the syndrome.

In the worst case, the affected person suffers a heart attack due to Churg-Strauss syndrome and can die from it. In the event of a heart attack, immediate medical treatment by an emergency doctor is required. Numbness or temporary paralysis can also indicate the disease.

Wounds that heal poorly or persistent abdominal pain and diarrhea should also be examined by a doctor to avoid further complications. The examination can be carried out by a general practitioner or by an ENT specialist . Furthermore, more precise imaging methods are necessary to make a conclusive diagnosis.

Treatment & Therapy

The disease is initially treated with antibiotics only . After that, corticosteroids are usually prescribed in low doses. These have an anti-inflammatory effect and are therefore mainly used in rheumatic diseases. The aim of the treatment is to preserve all organ functions by suppressing the signs of inflammation and, associated with this, normalizing the blood values.

If the heart or the peripheral nervous system is affected in Churg-Strauss syndrome, the doctor will administer an immunosuppressant . If left untreated, the disease leads to further spread of inflammation in the body’s vessels and thus carries the risk of blocking and cutting off the oxygen supply to vital body cells and organs. Close checks are always necessary for the early detection of relapses.

The earlier a recurrence is detected, the faster the doctor can intervene with less aggressive medication. If, after a long-lasting improvement, rheumatic complaints, increasing asthma and a general feeling of illness occur, these can be warning symptoms for a relapse.

Since Churg-Strauss syndrome is a rare inflammatory immune disease, patient and family information is a particularly important part of therapy. Patient training courses for vascular diseases are offered by various medical centers or the regional associations of rheumatic diseases.

Since the diagnosis of the disease often means a major cut in social, professional and family terms for those affected and their relatives, competent and holistic medical care is indicated. Sometimes accompanying psychotherapy can also be necessary in order to be able to deal with the diagnosis and the consequences of the disease.

The survival rate of those affected over a period of five years is 60 percent. It plays a major role here whether the vessels of the heart are affected by foci of inflammation.

Outlook & Forecast

Churg-Strauss syndrome does not heal itself. As a rule, the symptoms only worsen if they are not treated, and in the worst case they can lead to the death of the person concerned. Those affected suffer from fever and pneumonia, which can also lead to other inflammations in the body. As it progresses, Churg-Strauss syndrome can also lead to a heart attack if not treated properly. Patients often feel numbness, severe abdominal pain or diarrhea.

If the syndrome is not treated, wound healing is significantly delayed and permanent paralysis can also occur . In most cases, Churg-Strauss syndrome can be treated well with medication. The course of the disease is positive, although those affected are dependent on long-term intake of immunosuppressants. If left untreated, Churg-Strauss syndrome can lead to the spread of further inflammation.

Even after successful treatment, patients are dependent on regular examinations in order to detect a relapse at an early stage. Whether the life expectancy of the affected person is reduced by the syndrome depends heavily on whether the heart is also affected by the disease or not.

prevention

Since Churg-Strauss syndrome is an inflammatory autoimmune rheumatic disease that is not caused by any other primary disease, no preventive measures can be taken. A healthy and balanced lifestyle with a balanced diet and physical exercise is important for patients. It is also important for those affected to know about the warning signs of a relapse in order to then seek medical treatment accordingly.

aftercare

Eosinophilic granulomatosis with polyangitis (EGPA), formerly known as Churg-Strauss syndrome, is relatively rare. The syndrome has three phases. Depending on the age at which phase one occurs and when the correct diagnosis is made, treatment and aftercare are coordinated.

Here it is important to consider both the aftercare of phase one and the development of the second phase of the disease. In this sense, follow-up measures often include preventive measures at the same time. However, there can be several years between the development of individual phases of eosinophilic granulomatosis with polyangitis (EGPA). In this respect, follow-up care can be possible at longer intervals in each phase of the disease.

The relatively short average survival time between diagnosis and phase three without medical treatment is problematic. At best, 25 percent of those affected survive untreated. For medically treated victims, the survival rate is 75 percent. The risk of serious cardiovascular diseases is greatly increased by vasculitis. That alone requires careful follow-up care.

Monitoring of corticosteroid therapy is necessary. In addition, in severe cases of Churg-Strauss syndrome, chemotherapeutic agents, interferon, immunoglobulins or procedures such as plasmapheresis can be used. Such treatment approaches also require monitoring and follow-up measures due to severe side effects and after-effects. Since new preparations are also being tested for Churg-Strauss syndrome, clinical studies help to gain a better understanding.

You can do that yourself

Churg-Strauss syndrome is a very rare inflammatory immune disease. Educating patients and their families is therefore an important part of therapy. There are a number of clinics, health centers and health insurance companies that offer training on vascular diseases. The state associations of the Rheumatism League are also involved in this area.

The chances of recovery for the patient are higher the earlier the disease is recognized and treated. If the inflammatory processes are already so advanced that severe damage to the lungs and heart has occurred, the chances of survival drop drastically. The best self-help measure is therefore to consult a competent doctor as soon as possible. In the early stages, the syndrome resembles an allergic rhinitis or mild asthma. Anyone who observes such symptoms again and again without knowing the causes for them should not downplay these symptoms, but should have them clarified by a doctor as soon as possible.

Antibiotics are regularly prescribed as part of the treatment of Churg-Strauss syndrome . Because these drugs also kill beneficial bacteria in the gut, diarrhea is often very severe. Those affected can prevent this often very stressful side effect by consuming probiotic foods such as yoghurt . Taking bacterial cultures directly is much more effective. Appropriate preparations are available in pharmacies and health food stores.

In naturopathy , a change in diet, in particular a vegetarian diet without milk and milk products, is recommended for inflammatory immune diseases.

Lisa Newlon
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Hello! I am Lisa Newlon, and I am a medical writer and researcher with over 10 years of experience in the healthcare industry. I have a Master’s degree in Medicine, and my deep understanding of medical terminology, practices, and procedures has made me a trusted source of information in the medical world.