CS syndrome is a rare disease that affects the lungs, skin and sometimes other organs. It is also known as allergic granulomatosis.
What are the forms of Churg Strauss syndrome?
The main forms of Churg Strauss syndrome are:
1. Asthma
2. Eosinophil infiltration into the lungs
3. Eosinophils in the blood
4. Vasculitis
What are eosinophils?
Eosinophils are a type of white blood cell that is often elevated in allergic diseases.
What is obtained by biopsy in CS syndrome?
Tissue biopsy in CS syndrome also shows inflamed blood vessels and granulomas (a cluster of mixed chronically inflamed cells).
What are the symptoms of Churg Strauss syndrome?
CS syndrome is separated into 3 separate stages, which may or may not be consecutive. Symptoms depend on the stage and organs involved.
Prodromal phase:
- respiratory symptoms, especially asthma, predominate
- allergic rhinitis may also occur
Secondary phase:
- Eosinophils (eosinophilia) in peripheral blood
- Eosinophil infiltration into the lungs and / or intestines
- Weight loss, fever, sweating, abdominal pain and diarrhea
Vascular phase:
- Any organ can be affected, most often the heart, but the lungs, kidneys, lymph nodes and muscles can be affected.
- Bloody diarrhea, blood in the urine, joint pain, inflammation of the heart, convulsions, coma and nerve damage in the skin
- Asthma symptoms usually decrease as the disease progresses
- Kožajezahvaćena u više od 2 trećine pacijenata:
What are the skin changes?
• Blisters, spotting, and nodules often with black central pits caused by tissue necrosis
• Changes are most common on the back of the arms or the front of the legs, especially on the elbows and knees. Sometimes they are located on the scalp or trunk.
• New wounds may appear for months.
• At least 50% of deaths from CS syndrome are due to heart disease.
What causes Churg Strauss syndrome?
The cause of CS disease is not known but the presence of asthma, eosinophilia and increased levels of immunoglobulin E suggest an allergic process.
In some patients, it is associated with sensitivity to pencilin
How is it proven?
- the disease is still in the research phase
- there is no specific blood test for CS syndrome but usually the following tests are done:
- a) complete blood tests
- b) rheumatic factors, antinuclear antibodies
- c) tests of kidney, liver and muscle function
- d) imaging examinations include X-rays of the lungs and sinuses and an ECG.
e) skin biopsy and kidney biopsy may show a combination of eosinophilia, vasculitis and granuloma.
How is it treated?
Liječenje se provodi sa:
Systemic corticosteroids are the primary therapy for CS syndrome. Mild disease can initially be treated with oral corticosteroids, but in more severe forms of the disease, most authors suggest starting therapy with intravenous corticosteroids. Steroid treatment increased patient survival from 50% at 3 years to 75% at 8 years. Therapy is usually long-lasting.
The patient’s response to corticosteroids is usually dramatic because only within 1-2 weeks can the level of eosinophils, immunoglobulins as well as the level of muscle enzymes be normalized. Corticosteroids can then be reduced as the disease subsides. Low doses of prednisone may continue to be given if residual (long-term) asthma is present or other chronic symptoms are present.
If vascular symptoms are uncontrolled or if a large dose of steroids is required, the immunosuppressive cyclophosphamidemay be used. It is given to improve the condition of the disease but also to reduce the possibility of recurrence of the disease.
Other drugs reported to be useful in CS syndrome are:
- Methotrexate
- Tumor necrosis factor (TNF) blockers, such as infliximaband etanercept
- Recombinant interferon (IFN) -alpha
- Rituximab
Key words: Churg Strauss syndrome, heart, eosinophilia, skin biopsy, systemic corticosteroids,
In short: Churg Straus syndrome is a rare disease characterized by a high incidence of eosinophils in the blood in which the affected organs can be blood vessels, heart, brain, lungs, kidneys, digestive tract and skin. It is proven by skin biopsy and treated with systemic corticosteroid therapy.
Did you know: The use of corticosteroids can also be a predisposition to osteoporosis because they damage bone mass. This can be a problem especially for menopausal women or for older men. The use of calcium supplements, vitamin D, hormone replacement therapy, and / or bisphosphate may help protect against bone mineral loss.
For My doctor: Perica Ante dr.med.dermatovenerolog
