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. 2021 Apr;36(4):927-937.
doi: 10.1007/s00467-020-04763-5. Epub 2020 Oct 12.

Progression of proliferative glomerulonephritis with monoclonal IgG deposits in pediatric patients

Paul Miller #  1 , Andrew Y Xiao #  1   2 , Vanderlene L Kung  3 , Richard K Sibley  1 , John P Higgins  1 , Neeraja Kambham  1 , Vivek Charu  1 , Colin Lenihan  4 , Amanda M Uber  5 , Elizabeth M Talley  5 , Neiha Arora  6 , Vighnesh Walavalkar  7 , Zoltan G Laszik  7 , Cynthia C Nast  3 , Megan L Troxell  8
Affiliations

Progression of proliferative glomerulonephritis with monoclonal IgG deposits in pediatric patients

Paul Miller et al. Pediatr Nephrol. 2021 Apr.

Abstract

Background: Proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) is a glomerular disease defined by non-organized glomerular deposits of heavy and light chain-restricted immunoglobulin and is rarely reported in children.

Methods: We characterized a series of nine pediatric patients from two academic centers with biopsy-proven PGNMID and additionally describe two patients with monotypic IgG in the setting of IgM deposition.

Results: Each patient presented with hematuria and/or proteinuria; however, only five had elevated serum creatinine. Prodromal or concurrent infection was identified in six patients, low C3 in five, and alternate complement pathway gene variants in two. No monoclonal serum proteins were identified in five tested patients. Seven patients had monotypic deposits composed of IgG3-λ, two showed IgG3-κ, and one each IgG1 and IgG3 with lambda dominance in the setting of IgM deposition. The glomerular pattern was predominantly mesangial proliferative or membranoproliferative glomerulonephritis (MPGN). Treatment and outcomes were variable; four patients have recent PGNMID diagnoses and therefore minimal follow up, one had relatively stable kidney function for over a decade, and six experienced kidney failure, with four receiving transplants. Recurrent deposits of the same isotype were identified in five of six transplanted kidneys, corresponding to three of four transplanted patients. One of these patients developed PGNMID recurrences in three separate kidney allografts over a 20-year disease course.

Conclusions: Our study emphasizes the need for upfront IgG subclass investigation in pediatric mesangial or MPGN with IgG deposition and monotypic or biased light-chain staining. Furthermore, this pediatric experience suggests expanded pathogenic considerations in PGNMID. Graphical abstract.

Keywords: Children; IgG subclass; MPGN; Proliferative glomerulonephritis with monoclonal immunoglobulin deposits; Recurrent glomerulonephritis; Transplantation.

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