RESEARCH - Early mortality in systemic vasculitis: relative contribution of adverse events and active vasculitis

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Ann Rheum Dis. Published Online First: 1 July 2009. doi:10.1136/ard.2009.109389

BMJ Publishing Group Ltd & European League Against Rheumatism.

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Extended Report

Early mortality in systemic vasculitis: relative contribution of

adverse events and active vasculitis

Mark A Little 1, Nightingale 2, Cees A Verburgh 3, Hauser

4, Kirsten De Groot 5, Caroline Savage 1, R W Jayne 6 and

Lorraine Harper 1*

1 Renal Institute of Birmingham, University of Birmingham, United Kingdom

2 Wolfson Centre, Queen Hospital, United Kingdom

3 Kennemer Gasthuis, Netherlands

4 University Hospital, Zurich, Switzerland

5 Klinikum Offenbach, Germany

6 Addenbrooke's Hospital, United Kingdom

Abstract

Objective: To contrast the effect of the burden of vasculitis activity

with the burden of adverse events on one-year mortality of patients

with ANCA-associated vasculitis (AAV).

Methods: We assessed outcome and adverse events in patients

prospectively recruited to 4 European AAV clinical trials. We included

data on 524 patients with newly diagnosed AAV. The burden of adverse

events was quantified using a severity score for leukopenia, infection

and other adverse events, with an additional weighting for follow up

duration. A " combined burden of events score (CBOE) " was generated for

each patient by summing the individual scores. Vasculitis severity was

quantified using BVAS and GFR.

Results: One-year mortality probability was 11.1%; 59% and 14% of

deaths were due to therapy associated adverse events and active

vasculitis respectively. Using regression analysis, infection

score (p<0.001), adverse event score (p<0.001), leukopenia score

(p<0.001) and GFR (p=0.002) were independently associated with

mortality. The risk of one-year mortality remained low (5%) with CBOE

scores <7, but increased dramatically with scores above this. Hazard

ratio for death with a CBOE>7 was 14.4 (95% CI 8.4 to 24.8). Age and

GFR were independent predictors of CBOE score.

Conclusions: The greatest threat to patients with AAV in the first

year of therapy is from adverse events rather than active vasculitis.

Accumulation of adverse events, monitored using this scoring method,

should prompt increased awareness that the patient is at high risk of

death.

http://ard.bmj.com/cgi/content/abstract/ard.2009.109389v1?papetoc

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