Granulomatosis with polyangiitis (Wegener's)

Granulomatosis with polyangiitis (GPA) is a relatively rare necrotizing vasculitis belonging to the so-called ANCA-associated vasculitides, in that circulating anti-proteinase-3 neutrophil cytoplasmic antibodies (c-ANCA) can be detected in the large majority of patients. The major clinical manifestations include necrotizing granulomatous lesions in the upper and/or lower respiratory tract, and glomerulonephritis. Although generalized necrotizing vasculitis involving both arteries and veins is a frequent indication of its systemic character, limited forms restricted to paranasal sinuses can also be observed. In the absence of suitable therapies, the disease can lead to death in a high percentage of patients. The introduction of glucocorticoids associated to cyclophosphamide has marked a milestone in the treatment of GPA. This combination has in fact been able to remarkably improve the previously ominous prognosis of these patients, resulting in a 10-years’ survival rate of approximately 75 %. More recently, additional immunosuppressive drugs such as azathioprine, methotrexate, and rituximab have been employed, with comparable or even better results.