Introduction

For many people with AAV, treatment has come a long way. Medicines like rituximab, avacopan and mepolizumab mean remission is now realistic for most. Still, too many patients face slow diagnoses, labels that don’t quite fit, and the worry of relapse or long-term damage. A new article in Nature Reviews Rheumatology takes aim at these gaps. Its central message is simple but ambitious: stop treating diagnosis, classification and prognosis as separate boxes. Connect them — and build care around one integrated, patient-centred framework.

Diagnosis is where the journey often stalls. AAV can look very different from person to person, and even the best blood tests are not always decisive. The authors argue that it’s time to move beyond piecing together isolated test results. Instead, they call for shared diagnostic criteria that bring symptoms, labs, imaging and biopsy findings into a single pathway. They also point to the next wave of tools — biomarkers and genetic or immunologic signals — that could help identify AAV earlier, including in people who are ANCA-negative. The aim is practical: faster recognition, earlier treatment, and less irreversible organ damage.

Classification is the second lever for change. Traditional labels — GPA, MPA and EGPA — remain useful, and the distinction between PR3-ANCA and MPO-ANCA clearly carries prognostic weight. The paper’s contribution is to stop choosing between these views. It recommends a combined model that uses both the clinical picture and ANCA specificity, because together they better reflect real biology and guide decisions about monitoring and therapy. The authors also urge the field to validate the 2022 ACR–EULAR criteria more widely — especially in under-represented populations in Africa, Asia and Latin America — and to start layering in genetic and molecular profiles so that subgroups with distinct treatment responses can be defined with confidence. This is how classification turns from a label into a tool for precision care.

Prognosis is where success should be measured. Survival has improved; the next goal is how people live. The review proposes a more complete way to track outcomes by bringing three perspectives together: disease activity (for example with BVAS), accumulated organ damage (VDI), and what patients themselves report about symptoms and daily life (AAV-PRO). Add to that biomarkers and genetics to anticipate relapse or complications, and include treatment-related harm — especially from long-term steroids — as part of the scoreboard. The result is a composite view that helps clinicians adjust therapy earlier and more precisely, with quality of life front and centre.

International collaboration

All of this adds up to a larger shift. The authors argue for care delivered in specialist vasculitis centres where nephrology, rheumatology, pulmonology, ENT and neurology work side by side — and where patients are partners, not passengers. They call for international collaboration and data sharing so that tools are refined quickly and tested fairly across diverse groups. And they want research outcomes to reflect what matters in everyday life, not only what changes a lab value.

The takeaway is hopeful and practical: progress won’t come from new drugs alone. It will come from a more connected way of understanding AAV — the right diagnosis sooner, a classification that matches biology, and a prognosis that plans for health and quality of life, not just survival.

Viewing the full article

Since our chairman is a co-author of this publication, you can view the full article here. However, please note that it is not an open-access article — it is read-only, and all copyrights remain with the publisher.

Reference: “Challenges in the diagnosis, classification and prognosis of ANCA-associated vasculitis.” Nature Reviews Rheumatology (2025). DOI: 10.1038/s41584-025-01306-w.