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Assessment of disease activity and damage in the ANCA-associated systemic vasculitides 

Assessment of disease activity and damage in the ANCA-associated systemic vasculitides
Chapter:
Assessment of disease activity and damage in the ANCA-associated systemic vasculitides
Author(s):

Joanna C. Robson

, Ravi Suppiah

, and Raashid A. Luqmani

DOI:
10.1093/med/9780199579655.003.0061
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date: 23 September 2020

1. Currently there is no available gold standard method of assessment of disease activity; inflammatory markers can be non-specifically raised with infection or malignancy, rises in ANCA titre do not reliably predict clinical relapse and imaging modalities such as PET or MRI are not helpful in small vessel vasculitis.

2. In ANCA-associated vasculitis, disease activity and damage are both measured using specific validated clinical tools.

3. Disease activity is measured using the BVAS, which is a formalized assessment of active vasculitis over nine specific organ systems (systemic, cutaneous, mucous membranes/eyes, ENT, chest, cardiovascular, abdominal, renal and nervous systems).

4. Disease damage is measured using the VDI, which includes 64 items over 11 organ systems. Damage can be secondary to the effects of acute vasculitis or its treatment, with items only recorded if they have been present for more than 3 months.

5. Significant impairments in health-related quality of life have been demonstrated in patients with ANCA-associated vasculitis using generic tools such as the SF-36. The OMERACT vasculitis working group have identified the need to develop a vasculitis-specific patient reported outcome.

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