Stratified Therapy on Pediatric AAGN

Wang Mo200 enrolled

Overview

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

Study Type

INTERVENTIONAL

Allocation

NON_RANDOMIZED

Purpose

TREATMENT

Masking

NONE

Enrollment

200

Conditions

Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis ANCA-Associated Glomerulonephritis

GlucoCorticoidDRUG

The high-risk group: methylprednisolone intravenous 15-30mg/kg/d (Max.500mg/d)\*3 days , with 2 courses, interval 5-7 days;Oral prednisone acetate 1.5-2mg/kg/d (Max. 60mg/d) during methylprednisolone impact interval and later stage, with the first reduction after 2-4 weeks. The dose was reduced once every 3 weeks in the first 3 months, and once every 4 weeks in the next 3 months, with a reduction of 5-10mg/ day each time; Rituximab：375 mg/m2/ time, once a week for 4 times;Intravenous cyclophosphamide 7.5-10mg/kg.d\*2 days(Max.500mg/d), once every 4 weeks, twice in total, at the 8th and 12th week.The medium-low risk group: methylprednisolone was the same dose as before, but only with 1 course, then followed by oral prednisone acetate 1.5-2mg/kg/d (Max. 60mg/d), divided into three times for 3 weeks, and then changed to morning daily; The dose was reduced as the high-risk group ;Rituximab was the same dose as before.

Eligibility

Sex: ALLMin age: 5 YearsMax age: 17 Years

Medical Language ↔ Plain English

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.

Outcomes

Primary Outcomes

Response rate (complete and partial response rate)

The rate of complete or partial response to ANCA vasculitis-associated nephritis (AAGN) was assessed at 6 months. Complete AAGN response was defined as: 1. Estimated glomerular filtration rate (eGFR) ≥60ml/min/1.73m2, or a decrease of less than 15% from baseline eGFR; 2. Proteinuria ≤0.3g/24 hours.Partial AAGN response was defined as: 1. Estimated glomerular filtration rate (eGFR) ≥30ml/min/1.73m2, or a reduction of less than 50% from baseline eGFR; 2. Decreased proteinuria\>Baseline 50% or 24-hour urinary protein quantification\<50mg/Kg.d.

Time frame: 6 months

Secondary Outcomes

The time to achieve complete and partial AAGN remission