Background Even though immunosuppressive treatment has dramatically improved patient survival in the last years, ANCA positive vasculitides continue to cause high morbidity and frequent relapses in as much as 50% of patients.
Objectives The aim of the study was to evaluate long term outcome of ANCA positive vasculitis patients and to identify potential adverse prognostic factors.
Methods 33 ANCA positive vasculitis patients (60±14 years, 57.6% males) were retrospectively followed in a single center for a mean period of 61±48 months. Clinical manifestations and laboratory findings, number of relapses, induction and maintenance treatment, as well as adequate response, were evaluated. Disease activity was assessed using the BVAS/WG score and disease damage by the use of VDI score. Statistical significance was set at P≤0.05.
Results Patients had a mean BVAS/WG of 7.1±2.8 at initial presentation, which subsequently improved (BVAS/WG 1.3±1.6). Most prevalent manifestations were general symptoms (91%), renal manifestations (87.9%), lower (75.8%) and upper respiratory tract involvement (48.5%). 42.4% of the patients had a relapsing course of their disease (56 disease exacerbations in 33 patients). Mean remission time between relapses was 34.7±25.3 months. Subsequent relapses tended to have a milder course than initial presentation (BVAS/WG 3.8±2.8 vs. 5.7±3.2, respectively, P<0.001) and a better outcome (BVAS/WG 0.5±0.9 vs. 1±1.4, respectively, P<0.001).
Induction treatment included, in descending order, oral steroids, IV cyclophosphamide, oral cyclophosphamide and rituximab, whereas 50.9% of patients also received prophylactic co-trimoxazole treatment. Induction treatment lasted 5.9±4.1 months. Maintenance regiments mainly included oral steroids in tapering doses, mycophenolate mofetil and azathioprine, along with co-trimoxazole, while a small percentage (6.5%) also received subsequent rituximab cycles. Adverse events occurred in 33.3% of patients, with infections being the commonest, but they were all mild and transient, apart from 1 death due to PCP infection. At the end of follow up, 60.6% of patients were at full remission, 27.3% presented with mild disease (BVAS/WG ≤3), 9.1% were relapsing and 1 died. Mean BVAS/WG at the end of follow up was 1.5±2.3 and mean VDI was 1.2±1.2.
Microscopic polyangiitis was associated with a worse outcome, as indicated by a worse mean BVAS/WG and a worse BVAS/WG score at the end of follow up, whereas eosinophilic granulomatosis with polyangiitis had the best outcomes, as measured by mean BVAS/WG and BVAS/WG at the end of follow up (P≤0.05). Finally, a worse BVAS/WG score at relapse and older patient age affected outcomes negatively. No correlations were observed between different treatments and patient outcome, whereas disease damage at the end of follow up did not correlate to any clinical characteristics or assigned treatments.
Conclusions Long term outcome of ANCA positive vasculitis patients has largely improved due to immunosuppressive therapies, and even though a large percentage of patients continue to relapse, long term activity and damage have significantly ameliorated. Microscopic polyangiitis and a higher BVAS/WG score during relapse are associated with a relatively worse outcome; however, no other aggravating factors have been identified, provided that adequate and immediate treatment is administered.
Disclosure of Interest None declared