Objective: To describe the main features at diagnosis and evolution over time of patients with localised granulomatosis with polyangiitis (L-GPA) compared to those of systemic GPA (S-GPA). Methods: EULAR definitions of L-GPA, i.e., upper and/or lower respiratory tract involvement, and S-GPA were applied to patients from the French Vasculitis Study Group Registry. L-GPA and S-GPA patients' characteristics at diagnosis and long-term outcomes were analyzed and compared.