Current and future prospects in the management of granulomatosis with polyangiitis (Wegener's granulomatosis)

Abstract

Ruth M Tarzi, Charles D PuseyRenal and Vascular Inflammation Section, Department of Medicine, Imperial College, London, UKAbstract: Granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis) is a multisystem autoimmune condition associated with anti-neutrophil cytoplasm antibodies. Management of GPA can be complex, owing to the sometimes fulminant and multisystem nature of the presentation, the age demographics of the affected population, and a significant incidence of disease relapse. In this paper, we discuss how some of the challenges in the management of GPA have been and continue to be addressed including: reducing the toxicity of induction therapy; developing biomarkers to determine who can safely stop maintenance immunosuppression; improving the efficacy of maintenance therapy for relapsing patients; managing localized disease; and management of disease and treatment-associated comorbidity. Consideration is also given to emerging therapeutics in the treatment of GPA.Keywords: anti-neutrophil cytoplasm antibody, vasculitis, immunosuppression, biologics