Current Rheumatology Reports

, Volume 14, Issue 6, pp 494–500

Damage Assessment in ANCA-Associated Vasculitis

VASCULITIS (LR ESPINOZA, SECTION EDITOR)

Abstract

Antineutrophil cytoplasm antibody associated vasculitis has been transformed from life-threatening conditions to chronic relapsing long-term diseases as a result of significant advances in immunosuppressive therapy. Although mortality still occurs, it is much less frequent, with an average 5-year survival of over 70 %. In the setting of chronic conditions, it becomes increasingly important to monitor the burden of disease in terms of both active inflammation requiring immunosuppression and chronic damage (scarring) from vasculitis and its treatment and associated comorbidity. The damage that accumulates in patients with vasculitis does not respond to immunosuppressive treatment. It is important to distinguish disease activity from disease damage to prevent unnecessary immunosuppression, but it is equally important to recognize damage for what it is, so that it can be addressed appropriately. Damage is an inevitable consequence of long-term vasculitis for over 80 % of patients, which should not surprise us given the severity of the original illness. There is potential value in measuring damage as a means of providing prognostic information. Using a quantified score such as the Vasculitis Damage Index (VDI) allows us to predict mortality. Patients with at least five items of damage on the VDI score have substantially worse mortality (7- to 11-fold worse risk), as compared with those with lesser amounts of damage. These findings should be taken into context when planning the management of patients with vasculitis, as well as in clinical trials of vasculitis. Disease damage is an important surrogate for long-term outcome in vasculitis, and studies should be designed to limit the amount of damage accumulating as a result of therapeutic intervention, rather than simply controlling disease activity, as is currently the aim in recent randomized controlled trials in vasculitis. Furthermore, careful cataloguing of damage, as well as disease activity items, provides much greater detail in describing and observing the long-term natural history of primary systemic vasculitis in patients treated with immunosuppressive agents who survive their initial disease process.

Keywords

Vasculitis ANCA Mortality Damage Organ failure Hypertension Prognosis 

Abbreviations

AAV

ANCA associated vasculitis

ANCA

Antineutrophil cytoplasm antibody

BVAS

Birmingham Vasculitis Activity Score

DAS28

Disease Activity Score for 28 joints

GPA

Granulomatosis with polyangiitis (GPA)

HAQ-DI

Health Assessment Questionnaire Disability Index

LDIQ

Lupus damage index questionnaire

MPA

Microscopic polyangiitis

RA

Rheumatoid Arthritis

SDI

SLICC/SLE damage index

SHS

Sharp/van der Heijde Score

SLE

Systemic lupus erythematosus

SLICC

Systemic lupus international co-operating clinics

VDI

Vasculitis Damage Index

Copyright information

© Springer Science+Business Media, LLC 2012

Authors and Affiliations

  1. 1.Nuffield Orthopaedic CentreOxfordUK
  2. 2.NIHR Musculoskeletal Biomedical Research UnitNuffield Orthopaedic CentreOxfordUK

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