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Rare disease
CASE REPORT
Successful management of proliferative glomerulonephritis with monoclonal immune deposits with combined immunosuppressive therapy
  1. Dipesh Maan1,
  2. Barbara Clark1,
  3. Mark Bunker2 and
  4. Swati Arora3
  1. 1 Medicine, Allegheny Health Network, Pittsburgh, Pennsylvania, USA
  2. 2 Pathology, Allegheny Health Network, Pittsburgh, Pennsylvania, USA
  3. 3 Nephrology, Allegheny Health Network, Pittsburgh, Pennsylvania, USA
  1. Correspondence to Dr Dipesh Maan, vsdomestic{at}gmail.com

Abstract

Proliferative glomerulonephritis with monoclonal immune deposits (PGNMID) is a newly described entity characterised by monoclonal IgG deposits consisting of single light chain isotype and single heavy chain subtype (IgG1-4) in the kidneys. We are presenting two cases of patients who presented with acute kidney injury and worsening proteinuria. Kidney biopsy showed membranoproliferative pattern. Special staining for subclass of IgG showed monoclonal IgG3-kappa (case 1) and IgG1-kappa deposits (case 2) suggestive of PGNMID. Workup for underlying infection, malignancy, monoclonal gammopathy was negative. Since pathogenesis of PGNMID involves clonal proliferation of B-cells, we treated both patients with rituximab along with steroids that led to improvement of proteinuria and renal function. We also reviewed current literature to assess efficacy of rituximab in treatment of PGNMID. However, a larger pool of patients and a longer follow-up period is required to establish a role of rituximab and steroids in the treatment of this disease entity.

  • acute renal failure
  • nephrotic syndrome

https://doi.org/10.1136/bcr-2018-225205

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    Footnotes

    • Contributors All authors listed on the manuscript have contributed sufficiently to the project to be included as authors. The case was initially seen by the nephrology fellow DM. BC was the attending on the patient 2. She helped with the review of current management strategies. SA was the attending for patient 1. SA suggested to write up these rare cases and has mentored the write up, assisted the fellow in formatting the tables, figures and the final draft of the case report. SA and DM have conducted review of literature for assessing efficacy of rituximab in native and transplant kidneys with PGNMID. MB is the pathologist who assisted in obtaining the biopsy slides and helped in reviewing the manuscript as well. Final approval of the version to be published was done by all the authors: DM, BC, MB and SA.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed.