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2017 ; 3
(1
): e000435
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Long-term patient survival in a Swedish population-based cohort of patients with
ANCA-associated vasculitis
#MMPMID28955485
Heijl C
; Mohammad AJ
; Westman K
; Höglund P
RMD Open
2017[]; 3
(1
): e000435
PMID28955485
show ga
OBJECTIVES: Patients with antineutrophil cytoplasmatic antibodies-associated
vasculitides (AAV) exhibit higher mortality than the general population. In the
current study, we assessed whether cluster affiliation based on clinical
presentation might predict mortality. METHODS: With case record review, the
outcomes for a population-based cohort of patients diagnosed with AAV in southern
Sweden (catchment area of 0.7?million inhabitants) between 1997 and 2010 were
assessed. Based on organ involvement at presentation, the cohort was stratified
into the following clusters: gastrointestinal, cardiovascular, non-renal, renal
with proteinase 3 (PR3) and renal without PR3. Cluster affiliation, demographics,
clinical and laboratory values at entry were tested as prognostic factors for
survival in multivariable models. RESULTS: 195 patients (98 female) with a median
age of 69 years (IQR 55-77) at diagnosis were included in the cohort. The median
time of follow-up was 4 years for the 98 patients (50%) who died during follow-up
and 11 years for those alive at end of follow-up. The 1-year, 2-year, 5-year and
10-year survival was 87%, 82%, 70% and 55%, respectively. Prognostic factors for
survival were sex, age, renal function and cluster affiliation. The mortality of
patients with AAV was significantly increased compared with the general
population except in the non-renal cluster. The cardiovascular and
gastrointestinal clusters showed the highest mortality. CONCLUSION: Even though
the mortality in patients with AAV is increased compared with the general
population this does not apply to patients without gastrointestinal,
cardiovascular or renal involvement at diagnosis. We suggest that the initial
clinical presentation is an important predictor for survival.