Specific serological diagnosis of viral hepatitis.

Abstract

<h3>Background</h3> This study describes characteristics and outcomes of AAV patients included in a nation-wide initiative collecting clinical and laboratory data since 2009. <h3>Methods</h3> 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. <h3>Results</h3> 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&lt;0.001), but significantly lower in patients with lung involvement compared to those without (p&lt;0.01). Significant differences were found in the estimated survival of patients stratified according to the disease severity at diagnosis (localized &gt; early systemic &gt; generalized &gt; severe renal). <h3>Conclusions</h3> 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. <i>Supported by the Czech Kidney Foundation</i> <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.5777