Prognosis and Outcomes of ANCA-Associated Vasculitis

  • David JayneEmail author
Part of the Rare Diseases of the Immune System book series (RDIS)


In the modern therapeutic era the outcome of systemic vasculitis has changed from a usually fatal course to a chronic relapsing disorder [1]. During this period, the ANCA vasculitides have been defined and have received the most research attention, especially concerning treatment regimens and the longer term consequences of disease. Achievement of initial disease control, and removal of a direct threat to life or further organ damage, is now considered routine and physicians are concerned with their patients’ longer term health, prevention of further disease flares and comorbidities, minimizing the toxic consequences of treatment, and improving quality of life. From a research perspective, this has meant development of disease state definitions and objective quantification of disease activity and long-term outcome parameters, such as damage and glucocorticoid toxicity [2, 3]. The Birmingham Vasculitis Activity Score (BVAS) and its subsequent modifications are routinely used for activity assessment, while the vasculitis damage index (VDI) has been developed to assess all-cause damage after the vasculitis diagnosis. Disease-specific quality-of-life tools are in development and approaches to economic evaluation are at an early stage [4, 5].


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© Springer Nature Switzerland AG 2020

Authors and Affiliations

  1. 1.Department of MedicineUniversity of CambridgeCambridgeUK

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