Fibrillary glomerulonephritis associated with limited scleroderma: a case report

Clin Nephrol. 2016 Apr;85(4):235-7. doi: 10.5414/CN108723.

Abstract

Fibrillary glomerulonephritis (GN) is a rare glomerular disorder that has been associated with monoclonal gammopathies, malignancies, chronic infections, and autoimmune disorders. We present the case of a 56-year-old woman with limited-type scleroderma and remote discoid lupus, evaluated for dipstick positive hematuria and preserved kidney function. Serologies were negative. Kidney biopsy revealed fibrillary GN. Her renal function and proteinuria remain stable 4 years after her initial diagnosis. This case is unusual both in its presentation and evolution, but mostly because it is the first reported case of fibrillary GN in association with limited type scleroderma.

Publication types

  • Case Reports

MeSH terms

  • Basement Membrane / pathology
  • Complement C1q / analysis
  • Complement C3c / analysis
  • Female
  • Follow-Up Studies
  • Glomerulonephritis / complications*
  • Glomerulonephritis / immunology
  • Hematuria / etiology
  • Humans
  • Immunoglobulin G / analysis
  • Immunoglobulin kappa-Chains / analysis
  • Immunoglobulin lambda-Chains / analysis
  • Kidney Glomerulus / pathology
  • Lupus Erythematosus, Discoid / complications
  • Middle Aged
  • Proteinuria / etiology
  • Scleroderma, Limited / complications*
  • Scleroderma, Limited / immunology

Substances

  • Immunoglobulin G
  • Immunoglobulin kappa-Chains
  • Immunoglobulin lambda-Chains
  • Complement C1q
  • Complement C3c

Supplementary concepts

  • Glomerulopathy with Giant Fibrillar Deposits