Long-term patient survival in ANCA-associated vasculitis.
BACKGROUND
Wegener's granulomatosis and microscopic polyangiitis are antineutrophil cytoplasm antibodies (ANCA)-associated vasculitides with significant morbidity and mortality. The long-term survival of patients with ANCA associated vasculitis treated with current regimens is uncertain.


OBJECTIVE
To describe the long-term patient survival and possible prognostic factors at presentation in an international, multicentre, prospectively recruited representative patient cohort who were treated according to strictly defined protocols at presentation and included the full spectrum of ANCA-associated vasculitis disease.


METHODS
Outcome data were collected for 535 patients who had been recruited at the time of diagnosis to four randomised controlled trials between 1995 and 2002. Trial eligibility was defined by disease severity and extent, covered the spectrum of severity of ANCA-associated vasculitis and used consistent diagnostic criteria. Demographic, clinical and laboratory parameters at trial entry were tested as potential prognostic factors in multivariable models.


RESULTS
The median duration of follow-up was 5.2 years and 133 (25%) deaths were recorded. Compared with an age- and sex-matched general population there was a mortality ratio of 2.6 (95% CI 2.2 to 3.1). Main causes of death within the first year were infection (48%) and active vasculitis (19%). After the first year the major causes of death were cardiovascular disease (26%), malignancy (22%) and infection (20%). Multivariable analysis showed an estimated glomerular filtration rate <15 ml/min, advancing age, higher Birmingham Vasculitis Activity Score, lower haemoglobin and higher white cell count were significant negative prognostic factors for patient survival.


CONCLUSIONS
Patients with ANCA-associated vasculitis treated with conventional regimens are at increased risk of death compared with an age- and sex-matched population.
Royal Berkshire Hospital, Renal Unit, London Road, Reading, Berkshire RG1 5AN, UK. oflossmann@doctors.org.uk
Oliver
O
Flossmann
Annelies
A
Berden
Kirsten
K
de Groot
Chris
C
Hagen
Lorraine
L
Harper
Caroline
C
Heijl
Peter
P
Höglund
David
D
Jayne
Raashid
R
Luqmani
Alfred
A
Mahr
Chetan
C
Mukhtyar
Charles
C
Pusey
Niels
N
Rasmussen
Coen
C
Stegeman
Michael
M
Walsh
Kerstin
K
Westman
D000328:Adult; D017677:Age Distribution; D000368:Aged; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000188:drug therapy / Q000401:mortality*; D004812:Epidemiologic Methods; D005260:Female; D005919:Glomerular Filtration Rate; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D012074:Remission Induction; D016896:Treatment Outcome
D007166:Immunosuppressive Agents