Stratified Therapy on Pediatric AAGN

Stratified Therapy on Pediatric AAGN Based on Risk Assessment-A Prospective, Multicenter, Open, Tendentious Matched Control Clinical Study

Clinical information of children with ANCA-associated nephritis admitted to Children's Hospital Affiliated to Chongqing Medical University and partner centers from January 1, 2023 to December 31, 2023 was collected:

To evaluate and compare the differences in survival, renal outcomes, and adverse reactions in children with ANCA-associated nephritis given different interventions according to the revised PARRG risk stratification, and to evaluate the superiority of ANCA-associated nephritis given according to the revised PARRG risk stratification.

(2) To evaluate the efficacy and safety of glucocorticoid combined with rituximab and cyclophosphamide as induction regimen in high-risk group and glucocorticoid combined with rituximab as induction regimen in children with ANCA-associated nephritis (AAGN) in low and middle risk group based on PARRG risk stratification

Inclusion Criteria:

• 1) Newly diagnosed AAGN patients with onset age of 5-17 years, complete renal biopsy and regular follow-up in the hospital; 2) All enrolled children met the following inclusion criteria: ANCA classification criteria: Patients who meet the 2007 European Medicines Agency (EMA) classification algorithm or the 2012 CHCC definition. Clinical classification includes: micropolyvasculitis (MPA), granulomatous polyvasculitis (GPA) and eosinophilic granulomatous polyvasculitis (EGPA);

AAGN diagnostic criteria: ANCA vasculitis combined with any of the following:

1. Hematuria: gross hematuria or microscopic hematuria: 3 times microscopic hematuria within 1 week: red blood cells &gt in urine routine; 3 / high magnification field (HP) or > 17 /ul (higher than normal value, can be adjusted according to the standard of the laboratory of each center);
2. proteinuria: meet any of the following: ① 3 times in 1 week urine routine protein positive; 2) 24h urinary protein quantification > 150 mg or urinary protein/creatinine (mg/mg)> 0.2; 3 urinary microalbumin higher than normal for 3 times in 1 week;
3. Renal insufficiency: increased serum creatinine > 10% base or decreased creatinine clearance > 25%.

Exclusion Criteria:

- 1) Patients who relied on dialysis for more than 1 month at the time of diagnosis, or the proportion of glomerular sclerosis ≥75% at the time of renal biopsy; 2) Patients with severe infection (such as diffuse peritonitis, severe pneumonia, cellulitis, active Epstein-Barr virus infection, active cytomegalovirus infection, hepatitis B virus infection, tuberculosis infection, fungal infection, etc.) and tumor.

3) Patients with other primary or secondary kidney diseases (such as IgA nephropathy, membranous nephropathy, anti-glomerular basement membrane nephritis, polycystic kidney disease, renal dysplasia, urinary tract malformation, etc.); 4) Parents or children refused to join the group.