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

Authors Tarzi RM, Pusey CD

Received 20 December 2013

Accepted for publication 15 February 2014

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

DOI https://doi.org/10.2147/TCRM.S41598

Checked for plagiarism Yes

Review by Single-blind

Peer reviewer comments 4

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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