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Membranous nephropathy: a rare renal manifestation of IgG4-related systemic disease

Nidhi Jindal1, Dhiraj Yadav, Christopher Passero

  • 1University of Pittsburgh, Department of Medicine, Pittsburgh, PA, USA.

Clinical Nephrology
|March 27, 2012
PubMed

Insights

IgG4-related systemic disease (ISD) can rarely manifest as membranous nephropathy (MGN). Early diagnosis and steroid treatment are crucial for managing ISD to prevent irreversible kidney damage.

Area of Science:

  • Nephrology
  • Immunology
  • Rheumatology

Background:

  • IgG4-related systemic disease (ISD) is a multisystem fibroinflammatory condition.
  • Renal involvement in ISD typically presents as tubulointerstitial nephritis.
  • Glomerular manifestations, such as membranous nephropathy (MGN), are rare but documented.

Observation:

  • A patient presented with edema, hematuria, and significant proteinuria (15 g/d).
  • Renal biopsy confirmed membranous nephropathy (MGN) with numerous IgG4-positive plasma cells.
  • Elevated serum IgG4 levels (750 mg/dl) and IgG4-positive plasma cells in multiple tissues supported the diagnosis of ISD.

Findings:

  • Despite initial prednisone treatment, the patient progressed to renal failure requiring dialysis.
  • Rituximab therapy was initiated but did not prevent the need for dialysis.
  • The case highlights a rare renal manifestation of ISD as MGN.

Implications:

  • ISD can present with diverse renal pathologies beyond tubulointerstitial nephritis.
  • Early diagnosis and prompt steroid therapy are critical for preserving renal function in ISD.
  • Rituximab may be a therapeutic option for steroid-resistant cases of ISD with renal involvement.

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