Mycophenolate Mofetil for Severe Iga Vasculitis Nephropathy in Children: An Observational Study

Authors

Dmitry Samsonov, Division of Nephrology, Department of Pediatrics, Maria Fareri Children's Hospital, New York Medical College, Valhalla, New York.
Anna Zolotnitskaya, Pediatric Nephrology Division, Children's Hospital at Montefiore, Albert Einstein College of Medicine, Bronx, New York.
Robyn Matloff, Division of Nephrology, Connecticut Children's, Hartford, Connecticut.
Tanya Pereira, Division of Nephrology, Department of Pediatrics, Maria Fareri Children's Hospital, New York Medical College, Valhalla, New York.
Sonia Solomon, Division of Nephrology, Department of Pediatrics, Maria Fareri Children's Hospital, New York Medical College, Valhalla, New York.Follow

Author Type(s)

Faculty

DOI

10.1016/j.xkme.2022.100534

Journal Title

Kidney Medicine

First Page

100534

Document Type

Article

Publication Date

10-1-2022

Department

Pediatrics

Abstract

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 m 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 m) 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 Immunosuppression was successfully withdrawn in all patients, and at last follow-up (mean 33.5 months), all patients except one remained in remission.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.

Recommended Citation

Samsonov, D., Zolotnitskaya, A., Matloff, R., Pereira, T., & Solomon, S. (2022). Mycophenolate Mofetil for Severe Iga Vasculitis Nephropathy in Children: An Observational Study. Kidney Medicine, 4 (10), 100534. https://doi.org/10.1016/j.xkme.2022.100534