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Collapsing glomerulopathy in collagen vascular-like disease

R Nasr1, C Johns, E Gertner

  • 11Division of Rheumatology, University of Minnesota Medical School, USA.

Lupus
|November 29, 2013
PubMed

Insights

Collapsing glomerulopathy (CG) can present with collagen vascular-like disease, marked by severe proteinuria and renal insufficiency. Rheumatologists should consider this podocytopathy in patients with serological abnormalities but few clinical symptoms.

Area of Science:

  • Nephrology
  • Rheumatology
  • Immunology

Background:

  • Collapsing glomerulopathy (CG) is a severe podocytopathy often linked to viral infections or defined collagen vascular diseases like lupus.
  • A subset of patients presents with serological abnormalities suggestive of autoimmune disease but lacks definitive clinical criteria.

Observation:

  • This study details four new cases of CG with collagen vascular-like disease and reviews 14 similar cases from the literature.
  • Patients typically present with severe nephrotic-range proteinuria and kidney failure resistant to standard treatments.

Findings:

  • These patients exhibit positive antinuclear antibodies (ANA) and other significant serological markers without clear clinical rheumatologic diagnoses.
  • Most affected individuals progress to requiring dialysis.
  • Mycophenolate mofetil (MMF) shows potential as a therapeutic option.

Implications:

  • Rheumatologists should be aware of CG as a potential diagnosis in patients with unexplained proteinuria and renal insufficiency.
  • Early recognition and appropriate consultation are crucial for managing this challenging podocytopathy.
Abstract

Keywords:
Collapsing glomerulopathyantinuclear antibodycollagen vascular diseasepodocytopathy

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