Lung biopsy confirmed AAV in three patients, renal biopsy in two patients and skin biopsy in one patient (Figures 3A-3D). Out of the six patients who underwent biopsy, three patients were diagnosed with C-ANCA positive vasculitis, while two patients had P-ANCA positive vasculitis and one had ANCA-negative EGPA. Here, PRTN3 is linked to eosinophilic granulomatosis with polyangiitis.