Abstract
Eosinophilic granulomatosis with polyangiitis (EGPA) is a type of systemic vasculitis with eosinophilia in the peripheral blood, which is preceded by bronchial asthma or allergic disease. EGPA is pathologically characterized by microangiopathy granulomatosis vasculitis. Vasculitis can be exacerbated and cause central nervous system and cardiovascular disorders and gastrointestinal perforation. Histological examination reveals eosinophil infiltration and granulomas in lesions in areas such as the lung, nervous system, and skin. Laboratory tests show inflammatory findings such as C-reactive protein (CRP) elevation, increased eosinophils, elevated serum IgE, and elevated myeloperoxidase-anti-neutrophil cytoplasmic antibodies (MPO-ANCA). MPO-ANCA is positive in approximately 40–70% of cases of this disease. EGPA is a necrotizing vasculitis that affects small- and medium-sized blood vessels; however, it differs from other types of ANCA-related vasculitis (such as microscopic polyangiitis and granulomatosis) because it is preceded by bronchial asthma and eosinophilia in the blood and tissues. Treatment with immunosuppressive agents such as steroids or cyclophosphamide depends on the Five Factor Score, which predicts the prognosis and severity of the condition. If the effect of appropriate treatment with steroids is insufficient, the anti-interleukin-5 antibody mepolizumab can be administered. The combination of mepolizumab with standard treatment leads to a significantly longer duration of remission, a higher proportion of patients who achieve sustained remission, and less steroid use than with a placebo.
Highlights
Eosinophilic granulomatosis with polyangiitis (EGPA) was described in 1951 by J
Inflammatory cytokines such as tumor necrosis factor-α, interleukin (IL)-1β, and IL-8/CXCL8 are produced in response to antigen stimulation, and vascular endothelial cell damage due to degranulation and immune complex deposition associated with neutrophil activation leads to necrotizing vasculitis [10,11]
This disease is characterized by a combination of vasculitis in organs throughout the body and organ damage associated with eosinophil infiltration
Summary
Eosinophilic granulomatosis with polyangiitis (EGPA) was described in 1951 by J. When an allergic patient is stimulated by antigens from the respiratory tract, airway mucosa, mast cells, macrophages, T cells, and eosinophils produce eotaxin, which mobilizes eosinophils and cytokines and activates eosinophils [4,5,6]. Eosinophils secrete major basic proteins that damage tissues from eosinophil granules, eosinophil peroxidase, and platelet-activating factors, which are involved in the exacerbation of bronchial asthma and lesions, leading to peripheral neuropathy and myocardial damage [7,8,9]. Inflammatory cytokines such as tumor necrosis factor-α, interleukin (IL)-1β, and IL-8/CXCL8 are produced in response to antigen stimulation, and vascular endothelial cell damage due to degranulation and immune complex deposition associated with neutrophil activation leads to necrotizing vasculitis [10,11]. IL-5 is involved in eosinophil recruitment [12,13,14]
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