Stratified Therapy on Pediatric AAGN (STPARS)

Wang Mo

Status and phase

Enrolling

Phase 4

Conditions

ANCA-Associated Glomerulonephritis

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis

Treatments

Drug: GlucoCorticoid

Study type

Interventional

Funder types

Other

Identifiers

NCT05969522

ANCA20230706

Details and patient eligibility

About

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

Enrollment

200 estimated patients

Sex

All

Ages

5 to 17 years old

Volunteers

No Healthy Volunteers

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:

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);
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;
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.

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.

Trial design

Primary purpose

Treatment

Allocation

Non-Randomized

Interventional model

Sequential Assignment

Masking

None (Open label)

200 participants in 4 patient groups

Control group-Low and medium risk group

Other group

Description:

The children with AAGN admitted to all cooperating units during 2017.01.01 to 2022.12.31 were followed up for more than 6 months, and the case data were complete. PARRG Classification (pediatric AAGN renal risk grade, pediatric AAV renal risk grade, PARRG) According to the PARRG grading method provided by the Affiliated Children's Hospital of Chongqing Medical University, the enrolled children with AAGN were classified into high risk group, medium risk group, low risk group, medium risk group, low risk combination and low risk group according to the two indexes of eGFR and percentage of glomerular sclerosis during renal biopsy. PARRG risk grading criteria: There are two risk factors for ESRD: (1) eGFR≤20ml/min/1.73m2 at renal biopsy; ② The proportion of glomerular sclerosis ≥30%; High risk resistance: meet 2 risk factors; Moderate risk group: only 1 risk factor; Low risk group: no above risk factors;

Treatment:

Drug: GlucoCorticoid

Control group-High risk group

Other group

Description:

Treatment:

Drug: GlucoCorticoid

Experimental group-Low and medium risk group

Experimental group

Description:

Newly diagnosed children with AAGN admitted to our partners during the period of 2023.01.01 to 2023.12.31. All children with AAGN were PARRG risk graded before treatment with RTX and CTX: PARRG Classification (pediatric AAGN renal risk grade, pediatric AAV renal risk grade, PARRG) According to the PARRG grading method provided by the Affiliated Children's Hospital of Chongqing Medical University, the enrolled children with AAGN were classified into high risk group, medium risk group, low risk group, medium risk group, low risk combination and low risk group according to the two indexes of eGFR and percentage of glomerular sclerosis during renal biopsy. PARRG risk grading criteria: There are two risk factors for ESRD: (1) eGFR≤20ml/min/1.73m2 at renal biopsy; ② The proportion of glomerular sclerosis ≥30%; High risk resistance: meet 2 risk factors; Moderate risk group: only 1 risk factor; Low risk group: no above risk factors;

Treatment:

Drug: GlucoCorticoid

Experimental group-High risk group

Experimental group

Description:

Treatment:

Drug: GlucoCorticoid