ArticleClinical journal of the American Society of Nephrology : CJASN2015
Association of Serum C3 Concentration and Histologic Signs of Thrombotic Microangiopathy with Outcomes among Patients with ANCA-Associated Renal Vasculitis.
Article in Clinical journal of the American Society of Nephrology : CJASN, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 58 papers.
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Who cites it
58 citing papers in PubMed, 81 citations in OpenAlex.
- Multimodal Profiling of Complement Expression and Association With Outcomes in ANCA-Associated Vasculitis With Glomerulonephritis.Kidney international reports · 2026Article
- Effective Management of a Rare Case of Pediatric ANCA-Associated Vasculitis With Rituximab and Mycophenolate Mofetil.The American journal of case reports · 2026Article
- Complement C3 in panvascular disease: a central integrator of immune signaling and vascular remodeling.Clinical science (London, England : 1979) · 2025Review
- The role of complement C3 deposition in ANCA-associated glomerulonephritis: a registry-based study of clinical, histopathological, and prognostic insights.BMC nephrology · 2025Article
- Advances in the treatment of ANCA-associated vasculitis.Nature reviews. Rheumatology · 2025Review
- Current Understanding of the Pathogenesis of ANCA-Associated Vasculitis and Novel Treatment Options Targeting Complement Activation.Life (Basel, Switzerland) · 2025Review
- Targeted Complement Treatments in Glomerulopathies: A Comprehensive Review.Journal of clinical medicine · 2025Review
- Biomarkers in ANCA associated vasculitis: clinical utility, pitfalls and their role in the outcomes assessment.Frontiers in immunology · 2025Review
- Childhood-Onset ANCA-Associated Vasculitis: From Genetic Studies to Advances in Pathogenesis, Classification and Novel Therapeutic Approaches.International journal of molecular sciences · 2024Review
- Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis in China: Epidemiology, Management, Prognosis, and Outlook.Kidney diseases (Basel, Switzerland) · 2024Review
- Author's reply to "Immune complex-mediated glomerulonephritis with ANCA positivity: what should nephrologists consider?"CEN case reports · 2024Article
- Investigation of Clinical Features and Association between Vascular Endothelial Injury Markers and Cytomegalovirus Infection Associated with Thrombotic Microangiopathy in Patients with Anti-Neutrophil Cytoplasmic Antibody (ANCA)-Associated Vasculitis: Case-Based Research.International journal of molecular sciences · 2024Article
- Advances in the Assessment and Treatment of Anti-Neutrophil Cytoplasmic Antibody-Associated Glomerulonephritis.Journal of inflammation research · 2024Review
- Low levels of complement factor C3 at diagnosis can predict outcome in antineutrophil antibody associated vasculitis.Journal of nephrology · 2023Article
- Serum Complement C4 Levels Are a Useful Biomarker for Predicting End-Stage Renal Disease in Microscopic Polyangiitis.International journal of molecular sciences · 2023Article
- Unmet needs in ANCA-associated vasculitis: Physicians' and patients' perspectives.Frontiers in immunology · 2023Review
- A case of Alport syndrome with pregnancy-related atypical hemolytic uremic syndrome and crescentic glomerulonephritis.Clinical nephrology. Case studies · 2023Article
- Circulating immune-complexes and complement activation through the classical pathway in myeloperoxidase-ANCA-associated glomerulonephritis.Renal failure · 2022Article
- Relationship Between Gender and 1-Year Mortality in ANCA-Associated Vasculitis Patients: A Single-Center Retrospective Analysis and Meta-Analysis.Frontiers in medicine · 2022Article
- The Complement System and ANCA Associated Vasculitis in the Era of Anti-Complement Drugs.Frontiers in immunology · 2022Review
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Authors and funding
12 authors at 2 institutions in 2 countries.
Funding
No grant is acknowledged in the PubMed record.
Abstract
BACKGROUND AND
objectivesComplement alternative pathway (cAP) activation has recently been recognized as a key pathogenic event in ANCA-associated vasculitis (AAV). cAP dysregulation is also a major determinant of thrombotic microangiopathies (TMA), which can in turn complicate AAV. We explored the prognostic significance of cAP activation and of histologic evidence of TMA in a cohort of patients with renal AAV. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: We studied 46 patients with AAV diagnosed between January 1990 and December 2011 at the Nephrology Unit of Parma University Hospital; 30 of them had undergone renal biopsy. We analyzed serum levels of C3 (sC3) and C4 (sC4) and, for 19 patients who had frozen plasma, plasma Bb and C5b-9 levels. We also reviewed all kidney biopsy specimens, specifically searching for histologic signs of TMA, and performed immunofluorescence or immunohistochemistry for C3d, C4d, Bb and C5b-9.
resultssC3 was below the lower limit of normal in 35% of the patients, whereas C4 was low in only 2%. Patients with low sC3 tended to be older (P=0.04) and to have lower eGFR at diagnosis (P=0.06). The median follow-up was 78 months (interquartile range, 18-135 months); 18 patients reached ESRD (10 of 14 and 8 of 26 in the low and normal sC3 groups, respectively). Death-censored renal survival was lower in the low sC3 group than in the normal sC3 group (log-rank test, P=0.01). Eight of the 30 patients who had undergone biopsy (27%) had histologic signs of TMA; these signs were more frequent in patients with low sC3 (5 of 10 versus 3 of 20; P=0.04). Notably, patients with histologic signs of TMA had a dramatically worse death-censored renal survival than patients without TMA (log-rank test, P=0.01), with ESRD occurring in 8 of 8 patients with TMA versus 8 of 22 patients without TMA.
conclusionsLow sC3 levels and histologic signs of TMA are associated with a poor renal prognosis in patients with AAV.
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