Abstract: FR-PO0948
ANCA Positivity Is Associated with a Necrotizing and Crescentic Phenotype in Pediatric Patients with Lupus Nephritis: A Chinese Cohort Study
Session Information
- Pediatric Nephrology: Genetic Diseases, Development, Neonatal Nephrology, Glomerular Diseases, and More
October 23, 2026 | Location: Exhibit Hall A, Convention Center
Abstract Time: 10:00 AM - 12:00 PM
Category: Pediatric Nephrology
- 1800 Pediatric Nephrology
Authors
- He, Jia, Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China
- Yin, Lei, Shanghai Children's Medical Center Affiliated to Shanghai Jiaotong University School of Medicine, Shanghai, China
- Pan, Xiaoxia, Shanghai Jiao Tong University Medical School Affiliated Ruijin Hospital, Shanghai, China
- Xu, Jing, Shanghai Jiao Tong University Medical School Affiliated Ruijin Hospital, Shanghai, China
Background
ANCA positivity has been described in lupus nephritis (LN). Limited evidence suggests ANCA-positive LN in adults may carry more severe necrotizing and crescentic lesions,but its clinical and pathologic significance in pediatric populations is poorly defined.
Methods
We retrospectively studied 110 pediatric LN cases (2016–2025). Serum ANCA was tested by indirect immunofluorescence. Clinicopathologic features and short-term renal outcomes were compared between ANCA-positive (ANCA+) and ANCA-negative (ANCA−) groups. Within ANCA+ cases, an AAV-like LN phenotype was defined a priori as ≥2 of: crescents >15%, segmental fibrinoid necrosis, thrombotic microangiopathy, or systemic vasculitic features.
Results
Of 110 patients, 67 (61%) were ANCA+ (66 P-ANCA, 1 C-ANCA). Compared with ANCA−, ANCA+ cases showed higher SLEDAI, more pulmonary involvement, lower C3/C4, enriched positive anti-dsDNA, and significant excesses of cellular/fibrocellular crescents and fibrinoid necrosis despite comparable ISN/RPS class distribution. Twenty-six ANCA+ patients (38.8%) met AAV-like phenotype criteria, exhibiting more interstitial inflammation but fewer wire-loop lesions and weaker C1q deposition — a partial pauci-immune profile, alongside impaired baseline renal function and lower one-year LN remission (Table1).
Conclusion
The AAV-like phenotype in pediatric ANCA+ LN combines severe necrotizing/crescentic injury and TMA with reduced classical immune-complex features (less wire-loop, lower heavy C1q), higher disease activity, impaired baseline renal function, and worse one-year LN remission — defining an overlap phenotype that argues for routine ANCA testing as a risk-stratification tool in pediatric LN.
Acknowledgment
We are grateful to Professor Anthony Chang from University of Chicago for his valuable insights and constructive suggestions that improved this work. We also would like to thank the pediatric rheumatology specialists at the Department of Rheumatology and Immunology, Shanghai Children’s Medical Center, for providing partial data on pediatric lupus patients and supporting this study.
Table 1. Pathologic and outcome contrast within ANCA-positive pediatric LN: AAV-like phenotype vs. non-AAV-like.
Funding
- Government Support – Non-U.S.