The autoimmune disease – Churg-Strauss syndrome
Churg -Strauss syndrome is named after physicians Jacob Churg and Lotte Strauss, who first described it in the early 1950s. The syndrome is a vascular inflammation. This belongs to the group of rheumatic diseases . It primarily affects the small and medium-sized veins and arteries at the end of the bloodstream.
Churg-Strauss syndrome is a rare disease.
There are one to two new cases per year and per million inhabitants. Churg-Strauss syndrome peaks between the ages of 40 and 50. Due to the permanent inflammation, the vessels suffering from the syndrome are destroyed.
The blood supply to the organs in the immediate vicinity is no longer sufficient.
Care should be taken, especially with diseased vessels around the heart and lungs. The risk of dying from a heart attack or heart failure increases. There are medications for Churg-Strauss syndrome. These promise no cure, but prevent the progression of the disease.
There are no precise indications of the causes of the disease. The majority of patients are allergic and asthmatic.
The clinical picture of Churg-Strauss syndrome
Granulomas form in diseased arteries and veins. As a result, the immune system fights proteins in the blood vessels . White blood cells see them as not belonging to the body. You try to destroy them. This causes a vascular occlusion by penetrating the vascular wall and attracting further inflammatory cells with the help of messenger substances. The body attacks the immune cells. It releases substances that dissolve the tissue. Inflammation directly affects organs. In the heart, a heart muscle inflammation can be triggered. In general, Churg-Strauss syndrome increases the risk of heart attacks.
Symptoms and Warning Signs
Churg-Strauss syndrome characteristically occurs in three phases
- The first signs are allergic asthma and runny nose.
- This is followed by inflammation of the digestive organ and lungs.
- Finally, the typical vascular inflammation occurs with the formation of granulomas.
The third phase (vasculitic phase) usually occurs three years after the onset of the first phase. It can also be delayed by decades.
In 97 percent of cases, asthma is a symptom. 70 percent of patients with Churg-Strauss syndrome have symptoms such as muscle pain, flu-like conditions, a general malaise, nausea and weight loss. Sinus inflammation occurs in 61 percent. Other symptoms include skin disorders, such as hives, purpura, and skin nodules. If the kidneys are affected, high blood pressure and kidney failure are possible. During the second phase mentioned above, inflammation of the pancreas, problems with the gastrointestinal tract and bleeding occur. Churg-Strauss syndrome damages individual nerves, resulting in pain, numbness and even paralysis.
The doctor diagnoses Churg-Strauss syndrome with a blood test. An increase in the number of eosinophilic granulocytes and a reduced number of hemoglobin (anemia) are clear signs.
Before the doctor does a blood test, there must first be a suspicion of the disease. Heart problems and inflamed paranasal sinuses that indicate damage to the heart muscle arouse suspicion. It is the same with symptoms of nerve damage and sores or bleeding on the skin.
In 60 percent of sick people, the doctor does not discover the antibodies that work against their own body structures. With computer tomography, magnetic resonance imaging and X-rays, the doctor finds the exact location of the Churg-Strauss syndrome. With this he also determines the most favorable place for a tissue sample.
How is Churg-Strauss syndrome treated?
The most important goal of therapy is to ensure the survival of the patient and to prevent organ damage. The blood levels should normalize and the signs of inflammation should be suppressed.
In the early stages of the disease, treatment is mainly via antibiotics . Corticoids are added to supplement. If the Churg-Strauss syndrome is more advanced, the therapy consists of the latter and immunosuppressants. A more effective measure is corticosteroid shock treatment. Here, the doctor administers high doses of the drug over a few days. If the patient’s condition does not improve, another option is treatment with immunoglobulin.
Once the Churg-Strauss syndrome stops progressing, the doctor slowly reduces the dose. There is a long-term treatment, since there is no chance of recovery from this disease. By constantly taking the medication, the body suppresses the syndrome. Despite successful treatment, neurological damage may remain.
Once Churg-Strauss syndrome is discovered, the chances of successful treatment are good. If the vessels supplying the heart or the heart muscle are affected, the chances are significantly worse. The risk of dying from a heart attack or acute heart failure is high.
Churg -Strauss syndrome is a rare disease . The typical symptoms also occur without a disease of the syndrome. This makes it all the more difficult to arouse suspicion.
http://www.rheuma-liga.de/fileadmin/user_upload/Dokumente/Mediencenter/Publikationen/Merkblaetter/3.13_Churg_Strauss.pdf
© fotoliaxrender – Fotolia.com
Medical note for our readers:
Our patient information explains medical topics in a simple and understandable way. Our information is not a substitute for a medical examination. Please see a specialist for diagnosis or treatment.
