Churg-Strauss syndrome (patient information)
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]
Overview
Eosinophilic granulomatosis with polyangiitis previously called Churg-Strauss syndrome is a small and medium-sized necrotizing vasculitis, with extravascular granuloma formation. The etiology is not known. However, various environmental factors, allergens, genetics, and drugs may play a role in triggering disease process by activating eosinophils, B and T lymphocytes and macrophages. The disease is characterized by the presence of asthma, peripheral eosinophilia, rhinosinusitis, peripheral neuropathy and multiple organ involvements including skin, GI tract, and kidney.
What are the symptoms of Churg-Strauss syndrome
Symptoms of eosinophilic granulomatosis with polyangiitis typically develops through three phases, include prodromal phase, eosinophilic phase, and vasculitis phase. Although these phases cannot be clinically distinguishable.
- Prodromal phase: Most common manifestations include sinusitis, allergic rhinitis and asthma.
- Eosinophilic phase: This phase is characterized by peripheral eosinophilia and infiltration of eosinophils in multiple organs. Most commonly involved organs are lung and GI tract.
- Vasculitic phase: This phase is characterized by small and medium-sized vasculitis and inflammatory granuloma formation. Granulomas can be either vascular or extravascular. Commonly involved organs include lungs, kidneys, skin, heart, and peripheral nerves.
What causes Churg-Strauss syndrome?
There are no established causes for eosinophilic granulomatosis with polyangiitis. However, various allergens, infections, vaccinations and drugs may act as a triggering agents, and are responsible for developing disease. Genetics may also play a role.