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Isolation, Characterization, And High Throughput Extracellular Flux Analysis of Mouse Primary Renal Tubular Epithelial Cells
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IgG4-related kidney disease.

Lynn D Cornell1

  • 1Division of Anatomic Pathology, Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota 55905, USA. cornell.lynn@mayo.edu

Seminars in Diagnostic Pathology
|October 17, 2012
PubMed
Summary

IgG4-related kidney disease involves IgG4-related disease (IgG4-RD) affecting the kidneys, most commonly as IgG4-related tubulointerstitial nephritis (IgG4-TIN). This condition often responds well to immunosuppressive therapy, showing distinct histopathologic features on biopsy.

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Area of Science:

  • Nephrology
  • Immunology
  • Pathology

Background:

  • IgG4-related disease (IgG4-RD) is a systemic immune-mediated condition with diverse organ involvement.
  • Renal involvement, termed IgG4-related kidney disease, is increasingly recognized.
  • IgG4-related tubulointerstitial nephritis (IgG4-TIN) is the most frequent renal manifestation.

Purpose of the Study:

  • To review the histopathologic and immunophenotypic patterns of renal involvement in IgG4-RD.
  • To correlate these patterns with clinical, radiographic, and serologic features.
  • To provide a comprehensive overview of IgG4-related kidney disease.

Main Methods:

  • Review of histopathologic findings in renal biopsies from patients with IgG4-RD.
  • Immunohistochemical analysis for IgG4-positive plasma cells.
  • Correlation of pathological findings with clinical presentations and diagnostic data.

Main Results:

  • IgG4-TIN characterized by plasma cell-rich infiltrate, increased IgG4+ plasma cells, interstitial fibrosis, and immune deposits.
  • Glomerular involvement can manifest as membranous glomerulonephritis, IgA nephropathy, or other glomerulonephritis patterns.
  • IgG4-related plasma cell arteritis is another observed renal manifestation.

Conclusions:

  • IgG4-related kidney disease encompasses various renal pathologies driven by IgG4-RD.
  • IgG4-TIN is the predominant form, typically responsive to immunosuppression.
  • Accurate histopathologic and immunophenotypic characterization is crucial for diagnosis and management.