Outcomes and compliance with standards of care in anti-neutrophil cytoplasmic antibody–associated vasculitis—insights from a large multiregion audit

Objectives: We aimed to conduct a large audit of routine care for patients with ANCA-associated vasculitis (AAV). Methods: We invited all 34 hospitals within one health region in England to undertake a retrospective case note audit of all patients newly-diagnosed or treated with Cyclophosphamide or Rituximab for AAV April 2013-December 2014. We compared clinical practice to the BSR guideline for the management of adults with AAV, and use of Rituximab to the NHS England commissioning policy and NICE Technology Appraisal. Results: We received data from 213 patients. Among 130 newly diagnosed patients, delay from admission to diagnosis ranged from 0-53 days (median 6, IQR 3-10.5) for those diagnosed as in-patients. BVAS score was recorded in 8% at diagnosis. Remission at 6 months was achieved in 83% of patients. 1-year survival was 91.5%. 130 patients received Cyclophosphamide for new-diagnosis or relapse. The correct dose of i.v. cyclophosphamide (within 100mg of the target dose calculated for age, weight and creatinine) was administered in 58%. 25% of patients had an infection requiring hospital admission during or within 6 months of completing their cyclophosphamide therapy. 76 patients received Rituximab for new-diagnosis or relapse. 97% patients met NHS England or NICE eligibility criteria. PJP prophylaxis (recommended in the summary of product characteristics) was given in only 65% of patients. Conclusion: We identified opportunities to improve care, including compliance with safety standards for delivery of cyclophosphamide. Development of a national treatment protocol / checklist to reduce this heterogeneity in care should be considered as a priority