We published the EULAR recommendations for the management of systemic vasculitis in 2009. We proposed and received a mandate from EULAR to revise the recommendations for the management of ANCA associated vasculitis. This project was done in partnership with the ERA-EDTA.
A team of 22 experts representing rheumatology, renal medicine, chest medicine, clinical immunology, internal medicine, ENT medicine, ophthalmology, vasculitis nursing and patient support group was formed. There was representation from 11 European countries and the USA. EULAR standardised operating procedures were followed throughout. 10 items for update and 5 new items for consideration were identified via a Delphic process. Search strings were created to identify 1347 papers. Quality scoring of manuscripts was performed using the Critical Appraisals Skills Programme. 44 papers were identified to inform the recommendations. Level of evidence was ascertained as per EULAR SOP. Statements were formed and graded for strength by the task force and by the membership of the European vasculitis society for ascertaining further validity. The level of evidence for EGPA was poor and is reflected by the low grade of recommendations.
Patients with AAV should have expert involvement in their care. A biopsy remains the gold standard for diagnosis and should be performed whenever clinically feasible. Cyclophosphamide or rituximab are the mainstay of remission induction but methotrexate and mycophenolate mofetil may be appropriate for a small number of cases. Major relapses should be treated as new – but rituximab maybe favoured over cyclophosphamide in this category. Consideration should be given to strengthening immunosuppression even for patients with minor relapses. Patients with severe renal disease or diffuse alveolar haemorrhage should be considered for plasma exchange. Remission can be maintained with either azathioprine or rituximab. In a small number of patients, methotrexate of mycophenolate mofetil may be appropriate. Remission maintenance should be continued for a minimum of 24 months after which tapering should be considered. Refractory disease should be managed at an expert centre. The role of follow-up monitoring, urological assessment, immunoglobulin monitoring, cardiovascular risk, educational needs & comorbidity assessment has also been discussed.
Disclosure of Interest None declared