Primary systemic vasculitis with severe α1-antitrypsin deficiency revisited.
Objectives: To study the clinical characteristics and epidemiology of the combination of primary systemic vasculitis (PSV) and severe alpha-1 antitrypsin (α1-AT) deficiency. Method: Patients with PSV [granulomatosis with polyangiitis (GPA) (Wegener's), microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss), and polyarteritis nodosa] were identified through d
