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

Authors Tarzi R, Pusey C

Received 20 December 2013

Accepted for publication 15 February 2014

Published 17 April 2014 Volume 2014:10 Pages 279—293


Checked for plagiarism Yes

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Ruth M Tarzi, Charles D Pusey

Renal and Vascular Inflammation Section, Department of Medicine, Imperial College, London, UK

Abstract: 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

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