Eosinophilic granulomatosis with polyangiitis (EGPA) – colloquially called Churg-Strauss syndrome (CSS) – (synonyms: allergic granulomatous angiitis; Churg-Strauss granulomatosis; ICD-10-GM M30. 1: Panarteritis with pulmonary involvement) refers to a granulomatous (roughly: “granule-forming”) inflammation of the small to medium-sized blood vessels in which the affected tissue is infiltrated (“wandered through”) by eosinophilic granulocytes (inflammatory cells). The inflammation of the blood vessels is triggered immunoreactively.
Eosinophilic granulomatosis with polyangiitis belongs to the group of ANCA-associated vasculitides (AAV). ANCA stands for anti-neutrophil cytoplasmic antibodies. ANCA-associated vasculitides are systemic diseases, i.e. they can affect almost all organ systems. Characteristic of eosinophilic granulomatosis with polyangiitis is pulmonary involvement in the form of asthmatic symptoms.
The disease is rare.
Sex ratio: Women are affected about twice as often as men.
Peak incidence: The disease occurs predominantly between the ages of 40 and 50; however, it also occurs in children or adolescents
The incidence (frequency of new cases) of eosinophilic granulomatosis with polyangiitis is about1-2 cases per 1,000,000 population per year.
Course and prognosis: The use of immunosuppressive therapy has significantly improved the life expectancy of those affected in recent years.Recurrences are frequent, so patients should be monitored closely. Risk factors for relapse include early cessation of glucocorticoid therapy and lower total cyclophosphamide dose/duration of therapy. Symptoms suggestive of relapse include rheumatic symptoms, increase in bronchial asthma, and increase in eosinophils (eosinophil granulocytes/white blood cells).
The 5-year survival rate for eosinophilic granulomatosis with polyangiitis is over 80% with optimal therapy. The most common causes of death are myocardial infarction (heart attack) and heart failure.
