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Proliferative glomerulonephritis with monoclonal IgG deposits in children and young adults

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Abstract

Background

Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) has been recognized as a distinct entity in recent years. To the best of our knowledge, all patients with PGNMID reported thus far were older than 20 years of age. We now report five cases of PGNMID in patients under 20 years of age.

Methods

The clinical database was searched for patients with native kidney biopsies from 9/2011 to 8/2017, and cases with a diagnosis of PGNMID were retrieved. Light microscopy specimens and immunofluorescence and electron microscopy images were revisited. Clinical data and kidney biopsy findings for patients under the age of 20 were recorded.

Results

Five (0.78%) of a total of 637 patients younger than 20 with native renal biopsies had a diagnosis of PGNMID, including three males and two females with an average age of 14 years old (range 10–19). All five patients presented with microscopic hematuria and proteinuria. Three patients were nephrotic and their C3 levels were low. All five cases showed a membranoproliferative pattern with abundant mesangial and subendothelial monoclonal IgG3 deposits (3 κ and 2 λ light chain, respectively). The patients were followed up to 56 months. Two patients had re-biopsies 28 and 18 months after initial diagnosis and both showed similar morphologic changes. Various treatments were attempted including prednisone, mycophenolate mofetil, tacrolimus, rituximab, and eculizmab, with mixed responses.

Conclusions

PGNMID does occur in children and young adults. Membranoproliferative glomerulonephritis pattern with monoclonal IgG3 deposits is a common feature. Despite various immunosuppressive treatments, the disease appears slowly progressive.

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Authors and Affiliations

  1. Department of Nephrology, The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan, China

    Guolan Xing

  2. Department of Nephrology, Cook Children’s Medical Center, Fort Worth, TX, USA

    Robert Gillespie & Badreldin Bedri

  3. Children’s Renal Center, Medical City Children’s Hospital, Dallas, TX, USA

    Albert Quan

  4. Renal Path Diagnostics at Pathologists BioMedical Laboratories, 2501 S. State Highway 121, Suite 1220, Lewisville, TX, 75067, USA

    Pingchuan Zhang & Xin J. Zhou

  5. Department of Pathology, Baylor University Medical Center, Dallas, TX, USA

    Pingchuan Zhang & Xin J. Zhou

Authors
  1. Guolan Xing
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  2. Robert Gillespie
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  3. Badreldin Bedri
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  4. Albert Quan
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  5. Pingchuan Zhang
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  6. Xin J. Zhou
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Corresponding authors

Correspondence to Guolan Xing, Pingchuan Zhang or Xin J. Zhou.

Ethics declarations

The study was approved by the Institutional Review Board of Baylor University Medical Center at Dallas and performed in accordance with the Declaration of Helsinki.

Conflict of interest

The authors declare that they have no conflict of interest.

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Xing, G., Gillespie, R., Bedri, B. et al. Proliferative glomerulonephritis with monoclonal IgG deposits in children and young adults. Pediatr Nephrol 33, 1531–1538 (2018). https://doi.org/10.1007/s00467-018-3949-8

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  • DOI: https://doi.org/10.1007/s00467-018-3949-8

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