Vasculitis syndromes articles from across Nature Portfolio

Autoimmune vasculitis can be heterogeneous in terms of immune cell involvement. Here the authors use a single cell transcriptomics approach to characterise a group of microscopic polyangiitis patients that could be split into two groups typified by monocyte or Interferon associated gene expression.

Giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are increasingly viewed as parts of a single disease spectrum. In this Review, the authors outline how stratification of patients with GCA–PMR spectrum disease using clinical, imaging and laboratory data can to help guide therapy.

This article presents the first Evidence-Based Guideline dedicated specifically to the diagnosis and management of eosinophilic granulomatosis with polyangiitis. The 16 statements and five overarching principles cover the diagnosis and staging, treatment, outcome and follow-up of eosinophilic granulomatosis with polyangiitis.

In this Review, the authors discuss vascular involvement in Behçet syndrome and how the unusual pathogenesis involving an impaired immune-inflammatory response influences the treatment approach, which differs from that of other vasculitides.

In a head-to-head phase III trial of two drugs that target IL-5 or its receptor, benralizumab was noninferior to mepolizumab for the induction of remission in patients with eosinophilic granulomatosis with polyangiitis.

In patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis in sustained remission, reinfusion with rituximab after B cell repopulation resulted in fewer clinical relapses than did reinfusion following serological ANCA flare.

Using multi-omics analyses, researchers have identified two distinct immunological phenotypes of microscopic polyangiitis, which could inform prognosis and personalized treatment.

Platelet activity and the formation of monocyte–platelet aggregates contribute to the production of IL-1β and the development of cardiovascular lesions in Kawasaki disease.

The rarity of various forms of vasculitis, as well as other rheumatic diseases, presents difficulties in studying the genetics of these diseases as well as for evaluating treatments. Might new approaches, such as joint genetic analyses and drug repurposing, provide opportunities to learn more about these diseases and identify new therapies and serve as a basis for basket clinical trials?

A new study identifies proteinase 3 as an important driver of granuloma formation in granulomatosis with polyangiitis and points to potential therapeutic approaches.