Fig. 1: Main clinical characteristics of EGPA on the basis of ANCA status.

The clinical manifestations of eosinophilic granulomatosis with polyangiitis (EGPA) are quite heterogeneous and their frequencies differ on the basis of anti-neutrophil cytoplasmic antibody (ANCA) status. Specifically, vasculitic features (for example, glomerulonephritis, peripheral neuropathy and purpura) occur more often in ANCA-positive patients, whereas eosinophilic features (such as cardiac involvement and gastroenteritis) are more frequent in ANCA-negative patients. The vasculitic and eosinophilic phenotypes, however, are not clearly separated, as most patients manifest an overlap between vasculitic and eosinophilic features.