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Abstract
Background Five Factor Score (FFS) has been used in evaluating prognosis in systemic vasculitis including GPA. A revised FFS was published in 2011.1
Objectives This study is aimed to investigate usefullness of revised FFS and its relationship with mortality in GPA.
Methods Totally 77 (F/M: 41/36) GPA patients from database of Vasculitis center was retrospectively analyzed. Demographic and clinical characteristics were recorded. Five factor score of patients were calculated. Patients who had been lost from follow up was evaluated by phone call (n=14). Survival analysis was done by Kaplan-Meier method.
Results Mean ages of patients were 50±15 years. Distribution of organ involvements were as; ear-nose –throat (ENT): 81.7%, pulmonary: 83.3%, renal: 59.5%, skin: 32.4%, musculoskeletal: 68.6%, eye: 23.3%. ANCA was positive in 74% of patients (cANCA/ pANCA:65%/14.7%). Most frequent treatment protocol in remission induction was corticosteroid + cyclophosphamide (75.5%). Rituximab was used in 12 (15.6%) patients.
Seven of patients with renal involvement (n=23) were died, whereas three patients had renal transplantation and four had been on hemodialysis therapy.
Totally 14 patients had died. Causes of mortality were co-infections in chronic renal failure (n=2), pulmonary hemorrhage (n=3), pulmonary hemorrhage and infection (n=2), gastrointestinal perforation (n=2), infective endocarditis (n=1), not known (n=4).
Survival analysis was done with 63 patients. Median survival time was 36 (1-264) months. One-year and three-year survival rate was 92% and 81%, respectively. Twenty-three (36.5%) of patients had follow-up ≥5 years. Survival rate was statistically different between groups regarding to FFS scores (Table, p=0.003, Figure). 3-year survival rate was higher in patients with ENT involvement compared to without (87% vs 58%, p=0.052).
Survival analysis according to Five Factor score
Conclusions Three-year survival rate of GPA patients in our Vasculitis center is 81%. Survival rate was lower in patients with higher FFS scores concordant with literature. According to our results, FFS was found useful in evaluating prognosis in GPA.
References
Guillevin L ve ark. The Five Factor Score revisited. Medicine 2011; 90:19-27.
Disclosure of Interest None declared