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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 withAAV(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 BVASat diagnosis was 17.96 +/- 7.82 and was significantly higher in nonsurvivors than in survivors (mean +/- SD20.0 +/- 8.14 versus 16.95 +/- 7.47, respectively;P< 0.001). The mean +/- SD1996FFSand 2009FFSwere 0.81 +/- 0.94 and 1.47 +/- 1.16, respectively, and were significantly higher in nonsurvivors than in survivors (mean +/- SD1996FFS1.17 +/- 1.07 versus 0.63 +/- 0.81 [P< 0.001] and 2009FFS2.13 +/- 1.09 versus 1.15 +/- 1.05 [P< 0.001], respectively). Mortality rates increased according to the different 1996FFSand 2009FFScategories. In multivariate analysis,BVAS, 1996FFS, and 2009FFSwere significantly related to death (P= 0.007,P= 0.020,P< 0.001, respectively), but the stronger predictor was the 2009FFS(hazard ratio 2.9 [95% confidence interval 2.4-3.6]). When the accuracy ofBVAS, 1996FFS, and 2009FFSto predict survival was compared in the global cohort,ROCanalysis yielded area under the curve values of 0.60, 0.65, and 0.74, respectively, indicating that 2009FFShad 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 amongBVASand 1996FFSwas modest (r = 0.49;P< 0.001) but higher than betweenBVASand the 2009FFS(r = 0.28;P< 0.001). Conclusion BVASandFFSare useful to predict survival inAAV, but the 2009FFShas the best prognostic accuracy at any point of the disease course.

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.

Varon de 55 anos, fumador y bebedor importante, con antecedentes de gastrectomia Billroth I por ulcus gastrico hacia 24 anos y colecistectomia por colelitiasis hacia 3 anos. Consulto por dolor abdominal y vomitos. En la exploracion estaba afebril, hemodinamicamente estable y con estigmas de hepatopatia cronica. En la analitica destacaba elevacion discreta de la amilasa serica. Una tomografia computarizada (TC) abdominal (fig. 1) evidencio una coleccion subfrenica

OBJECTIVES: A potential point of concern among clinicians is whether results derived from the clinical trials can be reasonably applied or generalised to a definable group of patients seen in real world. It can be the case of the GiACTA study that is a phase III randomised controlled trial of tocilizumab (TCZ) in giant cell arteritis (GCA). To address this question, we compared the clinical features and the responses to TCZ from the GiACTA trial patients with those from a series of GCA seen in the daily clinical practice. METHODS: Comparative study of clinical features between patients from the GiACTA trial (overall n=251) and those from a multicentre series of real-world GCA patients undergoing TCZ therapy (n=134). The diagnosis of GCA in the GiACTA trial was established by the ACR modified criteria whereas in the series of real-world patients it was made by using the ACR criteria, a positive biopsy of temporal artery or the presence of imaging techniques consistent with large-vessel vasculitis in individuals who presented cranial symptoms of GCA. GiACTA trial patients received subcutaneous TCZ (162 mg every 1 or 2 weeks) whereas those from the clinical practice series were treated using standard IV dose (8 mg/kg/month) or subcutaneous (162 mg/week). RESULTS: Real-life patients undergoing TCZ were older with longer disease duration and higher values of ESR and had received conventional immunosuppressive therapy (mainly methotrexate) more commonly than those included in the GiACTA trial. Despite clinical differences, TCZ was equally effective in both GiACTA trial and clinical practice patients. However, serious infections were more commonly observed in GCA patients recruited from the clinical practice. CONCLUSIONS: Despite clinical differences with patients recruited in clinical trials, data from real-life patients confirm the efficacy of TCZ in GCA.