The aim of this study was to describe the clinical characteristics of ANCA-associated vasculitides (AAV) at presentation, in a wide cohort of Spanish patients, and to analyze the impact of the ...vasculitis type, ANCA specificity, prognostic factors, and treatments administered at diagnosis, in the outcome.A total of 450 patients diagnosed between January 1990 and January 2014 in 20 Hospitals from Spain were included. Altogether, 40.9% had granulomatosis with polyangiitis (GPA), 37.1% microscopic polyangiitis (MPA), and 22% eosinophilic granulomatosis with polyangiitis (EGPA). The mean age at diagnosis was 55.6 ± 17.3 years, patients with MPA being significantly older (P < 0.001). Fever, arthralgia, weight loss, respiratory, and ear-nose-throat (ENT) symptoms, were the most common at disease onset. ANCAs tested positive in 86.4% of cases: 36.2% C-ANCA-PR3 and 50.2% P-ANCA-MPO. P-ANCA-MPO was significantly associated with an increased risk for renal disease (OR 2.6, P < 0.001) and alveolar hemorrhage (OR 2, P = 0.010), while C-ANCA-PR3 was significantly associated with an increased risk for ENT (OR 3.4, P < 0.001) and ocular involvement (OR 2.3, P = 0.002). All patients received corticosteroids (CS) and 74.9% cyclophosphamide (CYC). The median follow-up was 82 months (IQR 100.4). Over this period 39.9% of patients suffered bacterial infections and 14.6% opportunistic infections, both being most prevalent in patients with high-cumulated doses of CYC and CS (P < 0.001). Relapses were recorded in 36.4% of cases with a mean rate of 2.5 ± 2.3, and were more frequent in patients with C-ANCA-PR3 (P = 0.012). The initial disease severity was significantly associated with mortality but not with the occurrence of relapses. One hundred twenty-nine (28.7%) patients (74 MPA, 41 GPA, 14 EGPA) died. The mean survival was 58 months (IQR 105) and was significantly lower for patients with MPA (P < 0.001). Factors independently related to death were renal involvement (P = 0.010), cardiac failure (P = 0.029) and age over 65 years old (P < 0.001) at disease onset, and bacterial infections (P < 0.001). An improved outcome with significant decrease in mortality and treatment-related morbidity was observed in patients diagnosed after 2000, and was related to the implementation of less toxic regimens adapted to the disease activity and stage, and a drastic reduction in the cumulated CYC and CS dose.
Objective
To compare the accuracy of the Birmingham Vasculitis Activity Score (BVAS), version 3, and the Five Factor Score (FFS), version 1996 and version 2009, to assess survival in antineutrophil ...cytoplasmic antibody–associated vasculitis (AAV).
Methods
A total of 550 patients with AAV (41.1% with granulomatosis with polyangiitis, 37.3% with microscopic polyangiitis, and 21.6% with eosinophilic granulomatosis with polyangiitis), diagnosed between 1990 and 2016, were analyzed. Receiver operating characteristic (ROC) curves and multivariable Cox analysis were used to assess the relationships between the outcome and the different scores.
Results
Overall mortality was 33.1%. The mean ± SD BVAS at diagnosis was 17.96 ± 7.82 and was significantly higher in nonsurvivors than in survivors (mean ± SD 20.0 ± 8.14 versus 16.95 ± 7.47, respectively; P < 0.001). The mean ± SD 1996 FFS and 2009 FFS were 0.81 ± 0.94 and 1.47 ± 1.16, respectively, and were significantly higher in nonsurvivors than in survivors (mean ± SD 1996 FFS 1.17 ± 1.07 versus 0.63 ± 0.81 P < 0.001 and 2009 FFS 2.13 ± 1.09 versus 1.15 ± 1.05 P < 0.001, respectively). Mortality rates increased according to the different 1996 FFS and 2009 FFS categories. In multivariate analysis, BVAS, 1996 FFS, and 2009 FFS were significantly related to death (P = 0.007, P = 0.020, P < 0.001, respectively), but the stronger predictor was the 2009 FFS (hazard ratio 2.9 95% confidence interval 2.4–3.6). When the accuracy of BVAS, 1996 FFS, and 2009 FFS to predict survival was compared in the global cohort, ROC analysis yielded area under the curve values of 0.60, 0.65, and 0.74, respectively, indicating that 2009 FFS had the best performance. Similar results were obtained when comparing these scores in patients diagnosed before and after 2001 and when assessing the 1‐year, 5‐year, and long‐term mortality. Correlation among BVAS and 1996 FFS was modest (r = 0.49; P < 0.001) but higher than between BVAS and the 2009 FFS (r = 0.28; P < 0.001).
Conclusion
BVAS and FFS are useful to predict survival in AAV, but the 2009 FFS has the best prognostic accuracy at any point of the disease course.
to compare the accuracy of Birmingham Vasculitis score (BVAS) v.3, and Five Factors Score (FFS) v.1996 and v.2009, to assess survival in ANCA-associated Vasculitis (AAV).
550 patients with AAV (41.1% ...GPA, 37.3% MPA, 21.6% EGPA) diagnosed between 1990-2016 were analyzed. ROC curves and multivariable Cox analysis were used to assess the relationships between the outcome and the different scores.
Overall mortality was 33.1%. The mean BVAS at diagnosis was 17.96±7.82, and was significantly higher in non-survivors than in survivors (20.0±8.14 vs. 16.95±7.47, p<0.001). The mean 1996FFS and 2009FFS were 0.81±0.94 and 1.47±1.16, respectively, and were significantly higher in non-survivors than in survivors (1.17±1.07 vs. 0.63±0.81, p<0.001; 2.13±1.09 vs. 1.15±1.05, p<0.001). Mortality rates increased accordingly to the different 1996FFS and 2009FFS categories. In multivariate analysis BVAS, 1996FFS and 2009FFS were significantly related to death (p=0.007, p=0.020, p<0.001), but the stronger predictor was the 2009FFS (HR 2.9, 2.4-3.6). When the accuracy of BVAS, 1996FFS and 2009FFS to predict survival was compared in the global cohort, ROC analysis yielded AUC values of 0.60, 0.65 and 0.74, respectively, indicating that 2009FFS had the best performance. Similar results were obtained when comparing these scores in patients diagnosed before and after 2001, and assessing the 1-year, 5-years and long-term mortality. Correlation among BVAS and 1996FFS was modest (r=0.49, p<0.001), but higher than between BVAS and 2009FFS (r=0.28, p<0.001).
BVAS and FFS are useful to predict survival in AAV, but 2009FFS has the best prognostic accuracy at any point of the disease course.
This is the first study comparing the BVAS, 1996FFS and 2009FFS accuracy to assess survival in patients with AAV, and the first to validate 2009FFS in these patients. This article is protected by copyright. All rights reserved.
Global brand management sometimes necessitates a decision as to whether to dub television advertisements, originally in English, into the first language of bilingual international markets. This ...research uses an experimental design to test the effectiveness of original English language and dubbed television advertisements among customers of a pharmaceutical company that is a global leader in the worldwide consumer healthcare market, and for which advertising represents one of the largest investments in its marketing budget. Results are provided, recommendations are made, limitations are noted and directions for future research are indicated. PUBLICATION ABSTRACT
PDF
