The Sound of Interconnectivity; The European Vasculitis Society 2022 Report

Abstract

The first European Vasculitis Society (EUVAS) meeting report was published in 2017. Herein, we report on developments in the past 5 years which were greatly influenced by the pandemic. The adaptability to engage virtually, at this critical time in society, embodies the importance of networks and underscores the role of global collaborations. We outline state-of-the-art webinar topics, updates on developments in the last 5 years, and proposals for agendas going forward. A host of newly reported clinical trials is shaping practice on steroid minimization, maintenance strategies, and the role of newer therapies. To guide longer-term strategies, a longitudinal 10-year study investigating relapse, comorbidity, malignancy, and survival rates is at an advanced stage. Disease assessment studies are refining classification criteria to differentiate forms of vasculitis more fully. A large international validation study on the histologic classification of anti-neutrophil cytoplasmic antibody (ANCA) glomerulonephritis, recruiting new multicenter sites and comparing results with the Kidney Risk Score, has been conducted. Eosinophilic granulomatosis with polyangiitis (EGPA) genomics offers potential pathogenic subset and therapeutic insights. Among biomarkers, ANCA testing is favoring immunoassay as the preferred method for diagnostic evaluation. Consolidated development of European registries is progressing with an integrated framework to analyze large clinical data sets on an unprecedented scale.

Keywords: clinical trials; genetics; histology; registries; vasculitis.

Figure 1

Trial map: Clinical trials informing on management of ANCA-associated vasculitis induction, maintenance, and relapse phases. The trial map summarizes key clinical trials conducted to refine induction of remission and maintenance of remission. Studies have included patients based on different “severity” of disease, with PEXIVAS including those presenting with severe kidney disease and pulmonary hemorrhage. Other studies such as the RAVE trial have excluded patients with the latter presentation form. Most maintenance studies in the past couple of years focused on rituximab as maintenance agent, and 1 trial has randomized only patients with relapsing disease course (RITAZAREM). All patients received rituximab as induction therapy and were then randomized to either rituximab or azathioprine as maintenance agent. Two trials have focused on long-term maintenance therapy, MAINRITSAN 3 and REMAIN. ANCA, anti-neutrophil cytoplasmic antibody.