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Complement profile in microscopic polyangiitis and granulomatosis with polyangiitis: analysis using sera from a
S Fukui1,2, K Ichinose1, K-E Sada3
1Department of Immunology and Rheumatology, Nagasaki University Graduate School of Biomedical Sciences , Nagasaki, Japan.
Scandinavian Journal of Rheumatology
|April 15, 2020
Summary
Serum properdin and factor D levels correlate with disease activity and damage in anti-neutrophil cytoplasmic autoantibody-associated vasculitis (AAV). These findings suggest distinct complement pathway involvement in microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA).
Area of Science:
- Immunology
- Nephrology
- Rheumatology
Background:
- The alternative pathway of complement is implicated in anti-neutrophil cytoplasmic autoantibody (ANCA)-associated vasculitis (AAV).
- Understanding the role of complement components in AAV pathogenesis is crucial for targeted therapies.
Purpose of the Study:
- To investigate the relationship between serum complement component levels and clinical characteristics in patients with microscopic polyangiitis (MPA) and granulomatosis with polyangiitis (GPA).
Main Methods:
- A nationwide prospective cohort study (RemIT-JAV-RPGN) measured serum levels of 15 complement components in 52 MPA and 39 GPA patients.
- Correlations with Birmingham Vasculitis Activity Score (BVAS) and Vasculitis Damage Index (VDI) were analyzed.
Main Results:
- Properdin levels were lower in MPA and GPA patients compared to healthy donors and negatively correlated with BVAS.
- Factor D levels positively correlated with VDI over time.
- Higher C5a/C5 ratio was associated with increased neutrophils, serum IgG, and lower creatinine.
Conclusions:
- Serum properdin and factor D levels are associated with disease activity and damage in MPA and GPA, respectively.
- Complement component profiles suggest pathological heterogeneity in MPA and GPA.
