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Background ANCA-associated vasculitis (AAV) presents clinically with variable severity of disease and current clinical guidelines give varying advice on initial treatment to induce remission. Achieving remission quickly is important to gain disease control and avoid cumulative organ damage from treatment related adverse events. Objectives This study examined real world practice of AAV treatment in Europe to understand the AAV severity spectrum and the response to therapy over 12 months. Methods 929 incident and 268 relapsing EU AAV patients receiving care from 399 physicians were studied. Patients were referred between 2014-17 and data collected retrospectively at baseline and 1, 3, 6 and 12 months following induction therapy. Birmingham Vasculitis Activity Score (BVAS) was collected in only 12% of patients and severity was defined as mild (localized disease with no systemic symptoms), moderate (Mod, systemic disease with lung and/or kidney involvement) or systemic (rapidly progressive systemic disease with lung and/or kidney involvement). Results AAV disease severity varied in both incident (mild 12.2%, moderate 54.3%, severe 33.6%) and relapsing (corresponding values 9.7, 64.6 and 25.7) patients at start of treatment. Comorbidity at time of induction therapy was more common in severe patients (71.8% of patients having at least one comorbidity) compared to mild patients (55.1%). Since BVAS was not measured routinely, clinical response was categorized as full (no vasculitis activity and GC taper on track), partial (reduction in vasculitis activity and major organ damage arrested) and no response (no improvement in vasculitis). Clinical response is presented below (% patients) for combination of incident and relapsing patients demonstrating that response varied with many patients having slow and/or incomplete response. Response varied by severity of the disease when induction therapy commenced. Conclusion Incident and relapsing AAV patients have variable disease severity at the time of induction therapy. Response to induction therapy is with few exceptions better in patients with milder AAV but overall many patients are slow to respond or have only a partial response to current induction therapy. Acknowledgement This work was supported by Vifor Pharma Disclosure of Interests Peter Rutherford Employee of: Vifor Pharma, Dieter Gotte Employee of: Vifor Pharma |
