REVIEW

Development of comprehensive disease assessment in systemic vasculitis

Oliver Flossmann¹, Paul Bacon², Kirsten de Groot³, David Jayne⁴, Niels Rasmussen⁵, Philip Seo⁶, Kerstin Westman⁷, Raashid Luqmani¹

¹ Botnar Research Centre, University of Oxford, Oxford, UK
² Department of Rheumatology, University of Birmingham, Birmingham, UK
³ Department of Nephrology, University of Lubeck, Lubeck, Germany
⁴ Department of Nephrology, Addenbrooke’s Hospital, Cambridge, UK
⁵ Department of Otolaryngology, Rigshospitalet, Copenhagen, Denmark
⁶ Vasculitis Center, Johns Hopkins University, Baltimore, Maryland, USA
⁷ Department of Nephrology, University of Malmo, Malmo, Sweden

Correspondence to:
Dr R Luqmani
Department of Rheumatology, Nuffield Orthopaedic Centre, Windmill Road, Oxford OX3 7LD, UK; raashid.luqmani{at}noc.anglox.nhs.uk

The systemic vasculitides are multisystem disorders with considerable mortality and morbidity and frequent relapses. In the absence of reliable serological markers, accurate clinical tools are required to assess disease activity and damage for treatment decisions, and for the performance of clinical trials. This article reviews and summarises the development and use of disease assessment tools for determining activity and damage in systemic vasculitis and reports ongoing initiatives for further development of disease assessment tools. A literature search was conducted using PubMed and reference lists for vasculitis, assessment, clinical trials, outcome and prognosis. The findings indicate that comprehensive disease assessment in vasculitis requires documentation of disease activity, chronic irreversible damage and impairment of function.

Abbreviations: ANCA, antineutrophil cytoplasmic antibody; BVAS, Birmingham Vasculitis Activity Score; BVAS/WG, Birmingham Vasculitis Activity Score for Wegener’s granulomatosis; DEI, Disease Extent Index; EUVAS, European Vasculitis Study Group; FFS, Five Factor Score; ITU, intensive treatment unit; OMERACT, Outcome Measures for Arthritis Clinical Trials; VDI, Vasculitis Damage Index

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