EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis

Abstract

In this article, the 2009 European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) have been updated. The 2009 recommendations were on the management of primary small and medium vessel vasculitis. The 2015 update has been developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS). The recommendations are based upon evidence from systematic literature reviews, as well as expert opinion where appropriate. The evidence presented was discussed and summarised by the experts in the course of a consensus-finding and voting process. Levels of evidence and grades of recommendations were derived and levels of agreement (strengths of recommendations) determined. In addition to the voting by the task force members, the relevance of the recommendations was assessed by an online voting survey among members of EUVAS. Fifteen recommendations were developed, covering general aspects, such as attaining remission and the need for shared decision making between clinicians and patients. More specific items relate to starting immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in lifethreatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, where licensed, in the setting of rapidly progressive renal failure or severe diffuse pulmonary haemorrhage. These recommendations are intended for use by healthcare professionals, doctors in specialist training, medical students, pharmaceutical industries and drug regulatory organisations.

ANCA-associated vasculitis

“…11,12 However, data are lacking on the precise duration of the maintenance regimen and recommendations have been made based on expert consensus. Early cessation of therapy (<1 year) is associated with an increased risk of relapse.…”

“…19 Hypogammaglobulinaemia has been observed after rituximab treatment and measurement of serum immunoglobulin levels prior to each course of rituximab and in those with recurrent infection is recommended. 12 Treatment intent is now long term and, therefore, attention should be paid to the morbidity associated with treatment. All patients should be screened for cardiovascular disease and appropriate treatment given to reduce risk factors.…”

“…10 However, because of the reduced total dose of cyclophosphamide associated with pulsed regimens, these are favoured in recent guidelines. 11,12 Mesna to reduce uroepithelial malignancy and haemorrhagic cystitis should be considered for all patients.…”

How to treat ANCA‑associated vasculitis: practical messages from 2016 EULAR/ERA‑EDTA recommendations

“…Generally, renal biopsies have a greater diagnostic yield compared to ENT biopsies. 2 Transbronchial biopsies have a much lower yield than open lung biopsies and are generally reserved for situations of diagnostic uncertainty where tissue may not be available from other sources. Biopsies also offer prognostic information.…”

“…The management should be either at or in conjunction with an expert center. 2 Glucocorticoid therapy is an extremely important adjunct for the management of AAV, but it is also responsible for the majority of adverse effects. It should be tapered down to 7.5 to 10 mg/d at 3 to 5 months.…”