Clinical features and structured clinical evaluation of vasculitis

• Published in: Best practice & research. Clinical rheumatology Vol. 32; no. 1; pp. 31 - 51
• Main Authors: Ponte, C., Águeda, A.F., Luqmani, R.A.
• Format: Journal Article
• Language: English
• Published: Netherlands Elsevier Ltd 01-02-2018
• Subjects: ANCA-Associated vasculitis; Damage; Disease activity; Disease assessment; Giant cell arteritis; Patient-reported outcomes; Prognosis; Quality of life; Takayasu arteritis; Vasculitis; Vasculitis; Disease activity; Prognosis; Giant cell arteritis; Takayasu arteritis; Patient-reported outcomes; Damage; ANCA-Associated vasculitis; Disease assessment; Quality of life
• ISSN: 1521-6942; 1532-1770
• DOI: 10.1016/j.berh.2018.10.001

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.