Background AAV are still related to a high morbidity and mortality in spite of therapeutic advances.To identify factors present at diagnosis that are associated with a worse prognosis, it is essential to tailoring the treatment

Objectives To describe the long-term survival rate and possible prognostic factors at presentation in a large cohort of patients with AAV from Spain, and compare BVAS a FFS prognostic scores

Methods Multicenter retrospective-longitudinal study that included patients diagnosed with AAV between Jan1995 and Dec 2013 in 20 Hospitals from Spain (REVAS Study). Statistical analysis was done using SPSS vs19.

Results Four hundred and fifty patients (50.4% men) were included: 184 GPA,167 MPA and 99 EGPA. ANCA were positive in 86.4% of cases:36.2% C-ANCA, 50.2% P-ANCA. Mean age at diagnosis was 55.6±17.4 y. Mean time to diagnosis was 4 weeks (0-240). The most frequent symptoms at diagnosis were fever (53%),constitutional symptoms (51%), arthralgia (53%), hypertension (29%), purpura (20%) and hemoptisis (19%). Renal failure was present in 47% of cases, lung involvement in 45.3%, pulmonary-renal syndrome in 16%,and cardiac involvement in 11%. Sensory peripheral neuropathy was detected in 16% of cases,mononeuritis multiplex in 28% and CVA in 4.5%. ENT was observed in 35% and eye involvement in 20%. All patients received oral glucocorticoids. Cyclophosphamide was given to 286 patients (45.3%IV and 38% oral). Dialysis was required in 19% of cases and plasma exchange in 7%. During the follow-up 40% of patients suffered bacterial infections, 10% sepsis 14.6% opportunistic infections and 6% neoplasms. One hundred and twenty-nine (28.7%) died: 74 (44.3%) with MPA,41 (22.3%) with WG and 14 (14%) with CSS. Mean survival was 82.5 months (0-307). Mortality was significantly higher in MPA (p=0.00). Poor prognosis factors at diagnosis were renal failure (HR 1.6),cardiac failure (HR 3.3),bacterial infections (HR 1.8)and age at diagnosis >65y (HR 4.1).BVAS and FFS were significantly associated with outcome.

Conclusions Among AAV, PAM is associated with the worse prognosis. Renal and cardiac failure, bacterial infections, and age at diagnosis >65y, are associated with a poor clinical outcome. Three of these factors are included in the revised version of FFS, supporting its usefulness as a prognostic score.

References

1. Flossmann O, Berden A, de Groot K et al. Long-term patient survival in ANCA-associated vasculitis. ARD 2011;70:488–94.

2. Guillevin L, C,Seror R,et al.The Five-Factor Score Revisited Assessment of Prognoses of Systemic Necrotizing Vasculitides. Medicine 2011;90:19-27.

Disclosure of Interest None declared