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Published on: October 13, 2023
Mycophenolate Mofetil for Severe IgA Vasculitis Nephropathy in Children: An Observational Study
Dmitry Samsonov1, Anna Zolotnitskaya2, Robyn Matloff3
1Division of Nephrology, Department of Pediatrics, Maria Fareri Children's Hospital, New York Medical College, Valhalla, New York.
Insights
Treatment with mycophenolate mofetil helps children with IgA vasculitis nephritis (IGAVN) achieve remission of nephrotic-range proteinuria. Proteinuria levels are key indicators for predicting remission time in these patients.
Area of Science:
- Pediatric Nephrology
- Immunology
- Glomerular Diseases
Background:
- IgA vasculitis nephritis (IGAVN) can lead to severe kidney complications like rapidly progressive glomerulonephritis and nephrotic-range proteinuria.
- These complications are associated with a poorer prognosis in affected children.
Purpose of the Study:
- To evaluate the efficacy of immunosuppressive treatment in children with IGAVN presenting with nephrotic-range proteinuria.
- To assess the time to remission, resolution of hematuria, and long-term kidney function.
Main Methods:
- Retrospective analysis of pediatric patients diagnosed with IGAVN, purpura, and nephrotic-range proteinuria between 2000 and 2018.
- Treatment involved steroids and mycophenolate mofetil; outcomes included proteinuria remission, hematuria resolution, and glomerular filtration rate (GFR) at 12 months and last follow-up.
Main Results:
- Twelve children (8 boys, 4 girls; mean age 7.5 years) were included. Initial mean urinary protein to creatinine ratio (UPC) was 12.5 mg/mg, and GFR was 90.7 mL/min/1.73 m².
- All patients achieved proteinuria remission (UPC <0.3 mg/mg) and normalized kidney function (GFR 102.2 mL/min/1.73 m²) within 12 months.
- At a mean follow-up of 33.5 months, all but one patient remained in remission, and all but one maintained normal GFR.
Conclusions:
- Mycophenolate mofetil-based immunosuppression is effective in achieving remission for children with IGAVN and nephrotic-range proteinuria.
- The magnitude of proteinuria is a significant predictor of the time required for remission.
- Long-term monitoring is essential for patients with severe IGAVN.
Rationale & Objective:
Individuals with IgA vasculitis nephritis (IGAVN) may develop rapidly progressive glomerulonephritis and/or nephrotic-range proteinuria, which are associated with worse prognosis. We report our experience of treatment of children with IGAVN with nephrotic-range proteinuria.
Study Design:
Case series.
Setting & Participants:
We retrospectively analyzed all children who presented with IGAVN, cutaneous purpura, and nephrotic-range proteinuria from January 1, 2000 until December 31, 2018.
Outcome:
We evaluated time required to achieve remission of proteinuria, resolution of hematuria, and glomerular filtration rate (GFR) at 12 months and last follow-up.
Results:
Twelve patients, 8 boys and 4 girls, mean age 7.5 years (range 4-15) were included in the study. Mean urinary protein to creatinine ratio (UPC) was 12.5 ± 8.7 mg/mg and GFR 90.7 ± 19.1 mL/min/1.73 m2 before initiation of immunosuppression. All patients were treated with steroids and mycophenolate mofetil. Mean UPC declined progressively from 12.5 mg/mg to 4.6, 2.7, 0.3, and 0.2 mg/mg after 1, 3, 6, and 12 months, respectively. All patients achieved remission of proteinuria (UPC <0.3 mg/mg) and normalization of kidney function (GFR 102.2 ± 8.0 mL/min/1.73 m2) at 12 months. Immunosuppression was successfully withdrawn in all patients, and at last follow-up (mean 33.5 months), all patients except one remained in remission. All patients except one that relapsed maintained normal GFR at the last follow-up.
Limitations:
Retrospective study, single-center experience, no standard immunosuppressive protocol, lack of control group.
Conclusions:
Remission can be achieved in patients with IGAVN and nephrotic-range proteinuria using mycophenolate mofetil-based immunosuppression. Magnitude of proteinuria is a key laboratory finding that correlates with time to achieve remission. Prolonged follow-up of patients with severe IGAVN is warranted.
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