Wegener’s Syndrome

Wegener's Syndrome, now more commonly referred to as Granulomatosis with Polyangiitis, is a systemic vasculitis characterized by necrotizing granulomatous inflammation affecting the respiratory tract and kidneys, often leading to renal failure. Its clinical significance lies in the potential for rapid deterioration if untreated, necessitating prompt diagnosis and immunosuppressive therapy. A key association includes the presence of anti-neutrophil cytoplasmic antibodies (ANCA), particularly PR3-ANCA.