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Eosinophilic granulomatosis with polyangiitis (EGPA), also known as Churg-Strauss syndrome, is a rare and often severe systemic vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs). EGPA can affect multiple organ systems, but the relationships between ANCA status and the organ-specific manifestations of EGPA in previous reports were inconsistent. To investigate the association of the ANCA status with organ-specific manifestations in EGPA. We performed a systematic review of studies published before March 16, 2020, in the PubMed, Embase,