Churg-Strauss syndrome is an inflammatory disease of the small blood vessels and belongs to the group of rheumatic diseases. Today, it is also known in medicine as eosinophilic granulomatosis with polyangiitis (EGPA). It was originally named by 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 primarily affected and have permanent foci of inflammation. Churg-Strauss syndrome is a primary vasculitis (inflammation of blood vessels); there is no other underlying physical disease causing these vascular inflammations. 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 with sufficient oxygen. Damage therefore occurs primarily 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 have asthma or another allergy. In addition to asthmatic symptoms, granulomas develop, which means nodular inflammatory cells accumulate in the small vessels of the lungs and skin. These are caused by the body’s own immune system turning against protein components of the blood vessel cells, fighting them off with the help of leukocytes and destroying them. This process is called autoimmune reaction. The immune system turns against the body’s own components and destroys them. In the vessel itself, the autoimmune reaction of the body leads to an inflammatory swelling of the vessel wall, which can completely close 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 occlusion of these can lead to a heart attack and thus death.
Symptoms, complaints and signs
The symptoms of Churg-Strauss syndrome are rather nonspecific at the beginning. Initially, most patients experience allergic asthma associated with allergic rhinitis. As the disease progresses, pneumonia with fever develops. If the heart is affected by the inflammatory lesions 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 may develop in the affected area. If the area around the stomach and intestines is affected, abdominal pain and diarrhea may occur. In the skin area, the disease manifests itself in the form of ulcers and poorly healing open wounds.
Diagnosis
If asthmatics experience other symptoms such as heart failure, pneumonia, recurrent inflammation of the sinuses, sores, and bleeding on the skin, the physician will order further examinations. For this purpose, 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, in almost half of the patients suffering from Churg-Strauss syndrome, certain antibodies can be detected which are directed against the patient’s own body structures. In general, the laboratory findings of the blood test show elevated levels of inflammation. X-rays, computed tomography, and magnetic resonance imaging can be used to find sites of inflammation in the heart, lungs, or sinuses.
Complications
Patients with Churg-Strauss syndrome suffer from constant inflammatory activity of the medium-sized and small arteries and the veins, which destroy the affected vessels. The result is an insufficient supply of blood to the organs located behind the damaged areas. Severe complications arise when the heart and lungs are affected. Damage to the 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. A characteristic feature of Churg-Strauss syndrome is the accumulation of inflammatory sites in nodular form, known as granulomas.This results in an autoimmune disease, with which the body’s own defense system is directed against individual components of the body. The inflammatory foci multiply and cause vascular occlusion. In addition to allergic asthma, patients suffer from rhinitis, breathing difficulties and chest pain. Mild pneumonia with fever may occur. Diarrhea, weight loss, and abdominal pain are accompanying symptoms in abdominal organ disease. If individual nerves are damaged, numbness occurs in the area of supply, leading to paralysis. If the heart is involved, the worst case scenario is inflammation of the heart muscle, heart failure or a heart attack. Treatment is by medication. The survival rate of patients with Churg-Strauss syndrome depends on prompt successful therapy and is 60 percent, although the involvement of the heart is crucial.
When should you see a doctor?
Unfortunately, Churg-Strauss syndrome does not present with particularly characteristic symptoms, so early diagnosis and treatment of this disease is not possible in many cases. A doctor should be consulted if the affected person suffers very frequently from rhinitis and possibly also from asthma and other breathing difficulties. Fever and inflammation in the lungs and ears may also indicate the syndrome. In the worst case, the affected person suffers a heart attack due to Churg-Strauss syndrome and may die from it. In case of an infarction, immediate medical treatment by an emergency physician is necessary. Furthermore, numbness or temporary paralysis may also indicate the disease. Wounds that heal poorly or persistent abdominal pain and diarrhea should also be examined by a physician to avoid further complications. The examination may be performed by a general practitioner or by an otolaryngologist. Furthermore, more accurate imaging is necessary to make a conclusive diagnosis.
Treatment and therapy
Initially, therapy of the disease is exclusively with antibiotics. Thereafter, corticosteroids are mostly prescribed in small doses. These have an anti-inflammatory effect and are therefore also mainly used in rheumatic diseases. The aim of treatment is to preserve all organ functions by suppressing the signs of inflammation and thus normalizing the blood values. If the heart or peripheral nervous system is affected in Churg-Strauss syndrome, an immunosuppressant is administered by the physician. If left untreated, the disease leads to the further spread of inflammatory foci in the body’s vessels, posing the risk of occlusion and cutting off the oxygen supply to vital body cells and organs. Close monitoring is always necessary for early detection of recurrences. The earlier a relapse is detected, the faster the physician can intervene with less aggressive medication. If rheumatic complaints, increasing asthma and a general feeling of illness occur again after a prolonged improvement, these can be warning symptoms of a relapse. Because Churg-Strauss syndrome is a rare inflammatory immune disorder, education of patients and family members is a particularly important component of therapy. Patient education for vascular disease is offered by various medical centers or the state associations of the Rheumatism League. Since the diagnosis of the disease often means a major break in social, occupational and family terms for those affected and their relatives, competent and holistic medical care is indicated. Sometimes, accompanying psychotherapy may also be necessary in order to cope 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 inflammatory foci.
Outlook and prognosis
In Churg-Strauss syndrome, there is no self-healing. As a rule, the symptoms only intensify if left untreated, and in the worst cases they can lead to the death of the affected person. The affected person suffers 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 experience numbness, severe abdominal pain or even diarrhea. If the syndrome is not treated, there will be a significant delay in wound healing and further also permanent paralysis. In most cases, Churg-Strauss syndrome can be treated well with the help of medication. The course of the disease is positive, but the affected persons are dependent on the long-term use 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 to detect a relapse at an early stage. Whether or not the affected person’s life expectancy is reduced by the syndrome depends largely on whether or not the heart is equally affected by the disease.
Prevention
Because Churg-Strauss syndrome is an inflammatory rheumatic autoimmune disease with no other underlying primary disease, no preventive measures can be taken. What is important for patients is a healthy and balanced lifestyle with a balanced diet and physical exercise. Furthermore, it is important for those affected to know about the warning signs of a relapse in order to seek medical treatment accordingly.
Aftercare
Formerly called Churg-Strauss syndrome, eosinophilic granulomatosis with polyangitis (EGPA) is relatively rare. The syndrome progresses in three phases. Depending on the age at which phase one occurs and when the correct diagnosis is made, treatment and follow-up are coordinated. Here, it is important to consider both the aftercare of phase one and the progression to phase two of the disease. In this sense, aftercare measures often include preventive measures at the same time. However, several years may elapse between the development of individual phases of eosinophilic granulomatosis with polyangitis (EGPA). In this respect, follow-up may be possible at longer intervals in each phase of the disease. The average relatively short survival time between diagnosis and phase three without medical treatment is problematic. Untreated, at best 25 percent of affected individuals survive. For medically treated sufferers, the survival rate is 75 percent. The risk of severe cardiovascular disease is greatly increased by vasculitis. This alone necessitates careful follow-up. Monitoring of corticosteroid therapy is necessary. In addition, chemotherapeutic agents, interferon, immunoglobulins, or procedures such as plasmapheresis may be used in severe cases of Churg-Strauss syndrome. Such treatment approaches also require monitoring and follow-up because of severe side effects and after-effects. Because new compounds are also being tested in Churg-Strauss syndrome, clinical trials help to improve understanding.
Here’s what you can do yourself
Churg-Strauss syndrome is a very rare inflammatory immune disorder. Therefore, educating patients and their families is an important part of therapy. There are a number of clinics, health centers, and health insurance companies that provide education about vascular disease. The regional 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 detected and treated. If the inflammatory processes have already progressed to the point where severe damage has been done to the lungs and heart, the chances of survival are drastically reduced. The best self-help measure is therefore to consult a competent physician as soon as possible. In the early stages, the syndrome resembles an allergic rhinitis or mild asthma. Anyone who repeatedly observes such symptoms without the causes being known should not play down these complaints, but should seek medical clarification promptly. Antibiotics are regularly prescribed as part of the treatment of Churg-Strauss syndrome. Since these drugs also kill beneficial bacteria in the intestine, very severe diarrhea often results. Patients can prevent this often very distressing side effect by consuming probiotic foods, such as yogurt. Much more effective is the direct intake of bacterial cultures. Corresponding preparations are available in pharmacies and health food stores. In naturopathy, a change in diet is recommended for inflammatory immune diseases, especially a vegetarian diet without milk and dairy products.
