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IgA nephropathy with minimal change disease.

Leal C Herlitz1, Andrew S Bomback2, Michael B Stokes3

  • 1Department of Pathology and Cell Biology, Division of Renal Pathology, and LB684@columbia.edu.

Clinical Journal of the American Society of Nephrology : CJASN
|April 12, 2014
PubMed
Summary

Rare IgA nephropathy with mild disease and nephrotic syndrome may mimic minimal change disease. These patients often show good response to treatment, suggesting a dual glomerulopathy presentation.

Keywords:
IgAnephrotic syndromerenal biopsyrenal pathology

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Area of Science:

  • Nephrology
  • Immunopathology
  • Glomerular Diseases

Background:

  • Immunoglobulin A nephropathy (IgA nephropathy) typically presents with hematuria and subnephrotic proteinuria.
  • Nephrotic syndrome is an uncommon manifestation of IgA nephropathy, usually linked to severe histological findings.

Purpose of the Study:

  • To characterize patients with IgA nephropathy presenting with nephrotic syndrome and mild histological disease.
  • To investigate the clinical presentation, biopsy findings, and outcomes in this rare subgroup of IgA nephropathy.

Main Methods:

  • Retrospective review of IgA nephropathy cases diagnosed between 2004 and 2011.
  • Inclusion criteria: nephrotic range proteinuria and histologically mild IgA nephropathy (lacking endocapillary proliferation or segmental sclerosis per Oxford Classification).

Main Results:

  • 17 patients identified with IgA nephropathy, nephrotic range proteinuria, and mild histology.
  • Biopsies showed mesangial proliferation and IgA deposits; electron microscopy revealed extensive foot process effacement.
  • 14/17 patients achieved complete response to treatment, with stable or improved renal function during follow-up.

Conclusions:

  • Mild IgA nephropathy with nephrotic range proteinuria may present similarly to IgA nephropathy with superimposed minimal change disease.
  • These cases likely represent a dual glomerulopathy, highlighting a distinct clinical entity within IgA nephropathy.