Eosinophilic granulomatosis with polyangiitis: modern view on pathogenesis and treatment (literature review)
Keywords:
eosinophilic granulomatosis with polyangiitis, Churg-Strauss syndrome, hypereosinophilic syndromes, asthmaAbstract
Eosinophilic granulomatosis with polyangiitis (EGPA), formerly known as Churg—Strauss syndrome is a rare systemic disease situated between primary small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies (ANCAs) and hypereosinophilic syndromes (HES). Clinical manifestations in EGPA tend to divide patients into two subsets of the disease, with a predominance of vasculitic or eosinophilic manifestations, and ANCA can differentiate between these two subsets. This subclassification contrasts ANCA-positive and ANCA-negative EGPA, but we have no insufficient understanding to use phenotype-tailored therapies. EGPA also remains a diagnostic problem because it can only be considered in the plane of allergic pathology for a long time. Asthma may be the primary or predominant manifestation for years, and the chronic corticosteroids requirement may mask other signs of the disease. Currently, immune dysregulation, genetic predisposition and clarify disease subcategorization are being actively studied, which can solve the problem of classification of EGPA.All of which will allow us to opportune diagnosis the disease and use specially adapted therapies.No conflict of interest was declared by the authors.
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