Membranous nephropathy with masked polyclonal IgG deposits associated with primary Sjögren's syndrome

CEN Case Rep. 2021 Feb;10(1):53-58. doi: 10.1007/s13730-020-00516-3. Epub 2020 Aug 7.

Abstract

Tubulointerstitial nephritis and renal tubular acidosis are well-known renal involvements with primary Sjögren's syndrome. However, several types of glomerulonephritis such as membranoproliferative glomerulonephritis and membranous nephropathy are also known to develop in patients with this syndrome. We here report a case of membranous nephropathy that developed 8 years after a diagnosis of primary Sjögren's syndrome in a female patient. Interestingly, the deposition was not identified by routine immunofluorescence using snap frozen tissue, but was revealed by immunofluorescence on formalin-fixed paraffin-embedded sections treated with proteinase K. We further performed immunofluorescence analysis on the treated paraffin-embedded sections with the identified antigen but found that the deposited IgG was not monoclonal and that serum amyloid P, a sensitive marker for membranous-like glomerulopathy with masked IgG κ deposits, was not evident in the glomeruli. To the best of our knowledge, this report depicted the first case of masked polyclonal IgG deposits and further analysis is needed to clarify the underlying mechanisms of IgG masking and possible association with autoantibodies.

Keywords: Membranous nephropathy; Membranous-like glomerulopathy with masked IgG-κ deposits; Sjögren’s syndrome.

Publication types

  • Case Reports

MeSH terms

  • Aged, 80 and over
  • Asian People / ethnology
  • Biopsy
  • Diuretics / administration & dosage
  • Female
  • Fluorescent Antibody Technique / methods
  • Glomerulonephritis, Membranous / drug therapy
  • Glomerulonephritis, Membranous / etiology
  • Glomerulonephritis, Membranous / immunology*
  • Glomerulonephritis, Membranous / pathology
  • Humans
  • Immunoglobulin G / immunology
  • Immunoglobulin G / metabolism
  • Kidney Glomerulus / immunology*
  • Kidney Glomerulus / pathology
  • Kidney Glomerulus / ultrastructure
  • Microscopy, Electron / methods
  • Sjogren's Syndrome / complications*
  • Sjogren's Syndrome / diagnosis
  • Treatment Outcome

Substances

  • Diuretics
  • Immunoglobulin G