Biomarkers in Primary Systemic Vasculitides: Narrative Review

TL;DR

This review explores current and emerging biomarkers for diagnosing and monitoring vasculitis, a group of inflammatory blood vessel disorders.

Contribution

The paper provides a comprehensive overview of validated and emerging biomarkers across different vasculitis subtypes.

Findings

Serum amyloid A (SAA) and interleukin-6 (IL-6) are key analytes reflecting systemic inflammation in vasculitis.

Promising biomarkers like PTX3 and MMP-9 are associated with large-vessel vasculitis.

Biomarkers such as Gd-IgA1 and AGT are relevant in IgA vasculitis, while TIMP-1 and S100 proteins are linked to ANCA-associated vasculitis.

Abstract

Vasculitides are a heterogeneous group of disorders characterized by inflammation of blood vessel walls, leading to tissue ischemia and organ injury. Traditional inflammatory markers such as the erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) are widely used but lack diagnostic specificity. This has driven the search for more informative biomarkers across vasculitis subtypes. This review summarizes current evidence for validated and emerging biomarkers in large-, medium-, small-, and variable-vessel vasculitis, as well as single-organ vasculitis. Key analytes reflect systemic inflammation, such as serum amyloid A (SAA) and interleukin-6 (IL-6), as well as endothelial activation, complement pathways, neutrophil and macrophage activation, and organ-specific damage. Promising candidates include pentraxin-3 (PTX3) and matrix metalloproteinase-9 (MMP-9) in large-vessel vasculitis; N-terminal pro-B-type natriuretic peptide (NT-proBNP) and S100 proteins in Kawasaki disease; galactose-deficient immunoglobulin A1 (Gd-IgA1) and urinary angiotensinogen (AGT) in IgA vasculitis; and tissue inhibitor of metalloproteinases-1 (TIMP-1), S100 proteins, complement C3, and PTX3 in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis. Although these biomarkers provide mechanistic insight, most lack disease-specificity, external validation, or standardized assays. Future progress will require multicenter studies, harmonized testing, and integrated biomarker panels combined with imaging modalities to improve diagnosis, activity assessment, and monitoring.

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