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Systemic vasculitides are a group of heterogeneous conditions with overlapping patterns of clinical and laboratory manifestations. Moreover, clinical features can be non-specific and seemingly disparate. A major factor in defining optimal therapy and measuring treatment response is careful disease assessment targeting four main domains: activity, damage, prognosis and quality of life/function. Assessment tools such as the Birmingham Activity Score and the Vasculitis Damage Index have become a core feature of clinical trials in ANCA-associated vasculitis (AAV) and formed the basis for sound clinical management of these complex conditions. We are still lacking accurate definitions of disease activity and damage progression in large-vessel vasculitis. There is an increasing interest in the role of patient-reported outcomes as a measure of disease impact; a disease-specific measure for use in AAV is being validated. We review how best to evaluate patients with large-, medium- and small-vessel vasculitis.

Original publication

DOI

10.1016/j.berh.2018.10.001

Type

Journal article

Journal

Best pract res clin rheumatol

Publication Date

02/2018

Volume

32

Pages

31 - 51

Keywords

ANCA-Associated vasculitis, Damage, Disease activity, Disease assessment, Giant cell arteritis, Patient-reported outcomes, Prognosis, Quality of life, Takayasu arteritis, Vasculitis, Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis, Disease Progression, Humans, Prognosis, Quality of Life