SP154
HUNGARIAN VASCULITIS REGISTRY – RESULTS OF THE FIRST FIVE YEARS Free

INTRODUCTION: Hungarian Vasculitis Registry was established in 2013 under auspices of the Hungarian Society of Nephrology. All Hungarian nephrology centers were asked to participate in data collection of patients with ANCA associated vasculitis. The primary aims were to increase the nephrologists’ vigilance about the disease, and to provide comparative epidemiological and outcome data.

METHODS: Anonymized patient data, collected using a custom-made web interface, includes patients’ demographics, initial symptoms, time elapsed from disease presentation until diagnosis, renal biopsy results by Berden classification, ANCA status, renal function, dialysis requirement and immunosuppression (ISU). Follow up data consists of disease activity, remission, relapse, renal function, need for renal replacement therapy (RRT) including transplantation, maintenance ISU, and cause of death.

RESULTS: Data from 334 patients was available for analysis. Mean age was 58.5±14.5 years, 64% were female, 34% had cANCA and 59% pANCA, 2% had both positivity and 5% proved to be ANCA negative. Renal biopsy was performed in 72% of cases. Prevalence of focal, diffuse crescentic, mixed and sclerotic histological classes were 28%, 36%, 29%, and 7%, respectively. Estimated GFR was 18.5±18.2ml/min/1,73m2 at baseline, and 31% of the patients required RRT. By induction ISU 24% of the initially dialyzed patients recovered renal function. Mean GFR for those who did not need RRT at diagnosis was 24.1±19.7ml/min/1,73m2, 15% of them developed ESRD during follow up. Predictors of RRT were diffuse, or mixed histology (OR 10.5, p<0.001 and OR 3.22, p=0.013, compared to focal, respectively) and the presence of skin lesions (OR 3.1, p=0.012). Presence of joint involvement seemed to be protective (OR 0.34, p=0.002).

Induction ISU included corticosteroid in 91.9%, cyclophosphamide in 83.2%, rituximab in 11%, and plasma exchange in 53% of cases. The most common maintenance treatment with corticosteroid was azathioprine (41%). Remission was achieved in 83% of patients.

During the median follow up of 30 months (IQR 6-78), 20% of patients died, 6% was transplanted and 6% was lost. One year, five year and ten year survival were 94%, 82%, and 69%, respectively. Long term survival appeared better in patients with cANCA compared to pANCA positive patients, but this difference disappeared by correcting for age. Predictors of death were age (HR 1.86, p<0.001, for 10 years increment) and requirement of dialysis (HR 2.98, p<0.001). Female gender (HR=0.52, p=0.024) and steroid maintenance therapy (HR=0.37, p=0.003) were protective factors. Relapse developed in 29% of patients, of which 21% occurred after 5 years. Risk of relapse decreased by age (OR 0.74, p=0.001), and plasma exchange as part of induction ISU (OR 0.54, p=0.019). The risk of relapse was higher with lower respiratory symptoms (OR=1.73, p=0.038).

CONCLUSIONS: As reflected by the increasing number of yearly reported patients, the Hungarian Vasculitis Registry helps growing nephrologists’ awareness of this rare disease and also provides opportunity for quality control. The documented epidemiological and clinical characteristics are comparable to other countries’ registry data, and underline the importance of early diagnosis to achieve the best outcome for our patients.