Background AAV are still related to a high morbidity and mortality in spite of new available therapies. To recognize factors associated with a worse prognosis is essential to tailor the treatment
Objectives To identify survival prognostic factors in a large cohort of patients with AAV from Spain and analyze changes in the new century
Methods multicenter retrospective-longitudinal study that included patients diagnosed with AAV between Jan95 and Nov14 in 20 Hospitals from Spain (REVAS Study). Statistical analysis was done using SPSS vs21
Results 455 patients were included: 188 (41.3%) GPA, 167 (36.7%) MPA, 100 (22%) EGPA. Mean age at diagnosis was 55.7±17.2 y. ANCA were positive in 86.8% of cases:35.8% C-ANCA and 51% P-ANCA. Median time to diagnosis was 4 weeks (IQR 10). Median follow-up was 80 months (IQR 105). Renal, lung, neurological and cardiac involvement were present in 56.7, 54.7%, 34.5% and 12.7% of cases,respectively. Pulmonary-renal syndrome was observed in 17%, ENT in 44.8% and eye involvement in 13.4%. Mean BVAS at diagnosis was 17.7±7.7. All patients received oral glucocorticoids; 342 (75%) cyclophosphamide (50.3% oral), and 6.6% biological therapy. Dialysis was required in 16.4% of cases and plasma exchange in 7%. During follow-up, 22.2% of patients suffered leucopenia, 39% bacterial infections, 12.7% opportunistic infections and 5.3% neoplasms. One hundred thirty (28.6%) patients died: 74 (44.3%) with PAM, 42 (22.3%) with GPA and 14 (14%) with EGPA (p<0.001). Median time to death was 57.5 months (IQR 105): significantly higher for EGPA (94m vs 69m for GPA and 50m for PAM;LogRank=0.01). Comparing diagnostic century, the percentage of deaths was higher in the XXth than in XXIth (48% vs 21%, p<0.001). No differences were found in clinical manifestations, but yes in treatment and rate of infections: in the actual century more plasma exchange is used (9% vs 2.2%, p=0.008), more biological therapy (9% vs 0.7%, p<0.001), less oral CF (29% vs 57.8%, p<0.001) and more CF IV pulses (52% vs 35.6%, p=0.001). Multifactorial Cox regression analysis showed that prognostic factors associated with survival were age>65y (HR 4.6), cardiac (HR 3.9) and renal involvement (HR 2.0). ENT involvement was a protector factor (HR 0.4). Bacterial infections during follow-up were also independent predictor of poor prognosis (HR 2.0)
Conclusions survival rate in AAV has significantly improved in the new century probably due to new treatment strategies, including less CF and biological therapy. Poor prognostic factors are age>65y, cardiac and renal involvement at diagnosis,and bacterial infections, while ENT involvement is protective.
Disclosure of Interest None declared