Background This study describes characteristics and outcomes of AAV patients included in a nation-wide initiative collecting clinical and laboratory data since 2009.
Methods Sixteen vasculitis centres (9x Nephrology, 4x Rheumatology, 2x Immunology, 1x Pediatrics) have participated in web-based data collection, consisting of retrospective data supplied at entry and prospective follow-up with a visit recorded every 3-6 months. Statistical analysis included the Kaplan-Meier method and log-rank test for survival analysis.
Results A total of 681 patients (M/F 326/355, median age at diagnosis 58, range 11-89 years) were enrolled (168pts are newly diagnosed), 338 (50%) c ANCA positive, 236 p ANCA (35%), 24 (4%) ANCA negative and 72 (11%) was not investigated. The mean time from diagnosis was 71 months (range 0-475). GPA was the most common diagnosis with 58%, followed by MPA (including renal-limited form) recorded in 35% and EGPA in 4%. Cumulative organ involvement involved kidney in 83% (confirmed with renal biopsy in 72% of them), lungs in 55% joint 35% and ENT in 34%. Most (88%) pts received cyclophosphamide, azathioprine was used in 60%,mycophenolate mophetil or acid in 24%, methotrexate in 2%, 35% of patients underwent PLEX. To cope with disease activity 35 pts (4%) were treated with biological therapies, most of them (27) by rituximab.
At present 512 pts are alive, pts status is assessed as complete remission in 72%, partial remission in 12%, persistent activity in 6%, primo-manifestation - new activity in 3% and relapse in 7%. Most (98%) pts. have hypertension (85% are treated by ACEi), 14 (2%) pts are post kidney Tx, 14% are dependent on dialysis. Last available VDI (Vasculitis Damage Index) ranged between 0 and 16 (median 4).
The estimated 5-year survival was 81.5% (95% CI =78.1% - 84.9%) and was significantly higher in patients with ENT involvement compared to those without (p<0.001), but significantly lower in patients with lung involvement compared to those without (p<0.01). Significant differences were found in the estimated survival of patients stratified according to the disease severity at diagnosis (localized > early systemic > generalized > severe renal).
Conclusions In the Czech population, GPA is the most common AAV, reflecting the northern European type.The disease severity at the diagnosisi is associated with higher mortality.
Supported by the Czech Kidney Foundation
Disclosure of Interest None declared