Churg-Strauss syndrome (Allergic granulomatosis)
Allergic granulomatous angiitis
Patients with Churg-Strauss syndrome is a disease that was previously considered a variant of polyarteritis nodosa (PAN) or Wegener's granulomatosis.
According to current doctrine it is, however, a distinct disease that is characterized by the following symptom triad:
- Granulomatous necrotizing vasculitis
- peripheral eosinophilia
- Asthma
The Origin of the Churg-Strauss syndrome is unclear to this day. Since there is an increased incidence of the disease after desensitization treatment and can often prove as eosinophils in the inflammatory areas and IgE antibodies, an autoimmune etiology is assumed.
Frequency
The Churg-Strauss syndrome is extremely rare. The incidence (incidence) is located in Great Britain for example from 1.7 to 5.9 per 1 million population per year.
Symptoms
- The symptoms are similar to the polyarteritis nodosa (PAN). There are, inter alia, arthralgias (joint pain) and palpable purpura (bleeding into the skin)
- In addition, lungs, heart, spleen, liver and gastrointestinal tract are particularly affected by vasculitis.
- Bronchial asthma
Diagnosis
The diagnosis of Churg-Strauss syndrome is difficult because often the asthmatic symptoms are important. On the diagnosis leading to the symptoms then are only coming abdominal discomfort and arthralgias.
- Evidence of eosinophilia and increased IgE titer in the laboratory
- Histologically also fall on the eosinophilia, and multiple small necrotic granulomas in the vicinity of the small vessels.
Therapy
Treatment is with corticosteroids, to which the disease usually responds very well
Forecast
The introduction of corticosteroid therapy was the 5-year survival rate increased the Churg-Strauss syndrome from almost 0% to 60-75%. Most common cause of death is a cardiomyopathy that leads to heart failure.