The Recommendations for the Management of ANCA-Associated Vasculitis (Part 1)
This week’s column discusses the European League Against Rheumatism (EULAR) recommendations for the management of antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV).
The recommendations, released in 2009, were focusing on the management of primary small and medium vessel vasculitis.
In contrast, the 2015 update was developed by an international task force representing EULAR, the European Renal Association and the European Vasculitis Society (EUVAS).
Evidence from systematic literature reviews and expert opinion, were the bases of the recommendation.
Experts presented, discussed and summarized the evidence in the course of consensus-finding and voting.
During the discussion, the levels of agreement (strengths of recommendations) were determined by the experts.
The importance of the recommendations was assessed through an online voting survey among members of the EUVAS and out of these, fifteen (15) recommendations were developed.
The recommendations cover aspects such as attaining remission and the need for shared decision-making between doctors and the patients.
The recommendations also placed special focus on immunosuppressive therapy in combination with glucocorticoids to induce remission, followed by a period of remission maintenance; for remission induction in life threatening or organ-threatening AAV, cyclophosphamide and rituximab are considered to have similar efficacy; plasma exchange which is recommended, especially taking into considerationthe rapidly progressing renal failure or severe diffuse pulmonary hemorrhage.
Granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) are collectively known as the antineutrophil cytoplasmic antibody (ANCA)- associated vasculitides (AAVs).
GPA, MPA and EGPA have respective annual incidence rates of 2.1–14.4, 2.4–10.1 and 0.5–3.7 per million in Europe, and the prevalence of AAV is estimated at to be 46–184 per million.
According to data, the five-year survival rates for GPA, MPA and EGPA are estimated to be 74–91%, 45–76% and 60–97%, respectively.
In 2009 the EULAR came up with recommendations for managing primary small and medium vessel vasculitis which included the management of AAV.
The update focused on treatment of AAV as well as using rituximab for AAV.
This update was made in cooperation with the European Renal Association—European Dialysis and Transplant Association (ERA-EDTA).
The recommendations present the standard therapy, the prognostic value of histopathology and management of long-term complications, integrating these into treatment methods.
Next week we will continue our discussion with the methods involving the recommendations and the standard therapy.