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Long-Term Outcome of ANCA-Associated Systemic Vasculitis

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Systemic Vasculitides: Current Status and Perspectives

Abstract

Long-term outcomes for patients with ANCA associated vasculitis have been transformed by treatment regimens including high dose glucocorticoids and cyclophosphamide; from a fatal disease in 80 % of patients in the 1950s, to a chronic disease with periods of remission and relapse in the majority in the last decade [1]. The focus now is on refining the regimens used, including the use of cyclophosphamide and glucocorticoid reduction strategies, to maximise effectiveness of remission induction and maintenance, and reduce irreversible damage accumulation, whilst minimising adverse effects including infection, cardiovascular disease and malignancy. A key element of the analysis of long-term outcomes has been the identification of prognostic markers for poorer outcomes (see Table 14.1) which may respond to targeted treatment regimens. One example is the response to Rituximab, particularly in relapsing disease which is PR3 ANCA positive [2]. The Outcome Measures in Rheumatology (OMERACT) Vasculitis Working group has spearheaded the development and use of outcome measurement within ANCA-associated vasculitis and defined a core set for use in clinical trials [3]. Outcomes including measures of disease activity and irreversible damage have enabled standardised disease assessments within clinical trials and enabled the description and analysis of long-term outcomes in a way that was previously difficult to do in what is a relatively rare disease. The OMERACT Vasculitis group is now also looking beyond the use of physician based outcome tools to the development of disease specific patient reported outcome measures to enable future treatment regimens to be assessed via outcomes that measure what is important to patients [4].

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Authors and Affiliations

  1. Academic Rheumatology Unit, The Courtyard, Bristol Royal Infirmary, BS2 8HW, Bristol, UK

    James Ritchie, Timothy Reynolds & Joanna C. Robson

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  1. James Ritchie
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  2. Timothy Reynolds
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Correspondence to Joanna C. Robson .

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  1. University of Bari Medical School, Bari, Italy

    Franco Dammacco

  2. University of Bari Medical School, Bari, Italy

    Domenico Ribatti

  3. University of Bari Medical School, Bari, Italy

    Angelo Vacca

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Ritchie, J., Reynolds, T., Robson, J.C. (2016). Long-Term Outcome of ANCA-Associated Systemic Vasculitis. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_14

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