Goodpasture's syndrome with concomitant immune complex mixed membranous and proliferative glomerulonephritis

Clin Nephrol. 2014 Mar;81(3):216-23. doi: 10.5414/CN107454.

Abstract

Classical Goodpasture's (GP) syndrome is a monophasic illness characterized by pulmonary hemorrhage and rapidly progressive glomerulonephritis with linear IgG deposition along the glomerular and distal tubular basement membrane and estructive necrotizing diffuse extracapillary crescentic glomerulonephritis. The majority of patients have circulating anti-glomerular basement membrane (GBM) antibodies, detectable with standard anti-GBM ELISA. Concurrence of GP syndrome with proliferative glomerulonephritis has only rarely been described. In this report, for the first time we describe in a 21-year-old woman GP syndrome with 50% crescentic sclerosing glomerulonephritis with linear immunofluorescence characteristic of anti-GBM pathogenesis, combined with mixed membranous and membranoproliferative glomerulonephritis with granular immunofluorescence and subepithelial, mesangial and subendothelial deposits characterizing immune complex pathogenesis. The clinical picture was also unusual for GP syndrome, manifesting a recurrent but non-progressive course, nephrotic syndrome, normal renal function and low values of anti-GBM antibodies, identified only by novel more sensitive techniques.

Publication types

  • Case Reports

MeSH terms

  • Anti-Glomerular Basement Membrane Disease / complications*
  • Anti-Glomerular Basement Membrane Disease / diagnosis
  • Anti-Glomerular Basement Membrane Disease / drug therapy
  • Anti-Glomerular Basement Membrane Disease / immunology
  • Autoantibodies / analysis*
  • Biomarkers / analysis
  • Biopsy
  • Drug Therapy, Combination
  • Female
  • Fluorescent Antibody Technique
  • Glomerulonephritis, Membranoproliferative / complications*
  • Glomerulonephritis, Membranoproliferative / diagnosis
  • Glomerulonephritis, Membranoproliferative / drug therapy
  • Glomerulonephritis, Membranoproliferative / immunology
  • Glomerulosclerosis, Focal Segmental / complications*
  • Glomerulosclerosis, Focal Segmental / diagnosis
  • Glomerulosclerosis, Focal Segmental / drug therapy
  • Glomerulosclerosis, Focal Segmental / immunology
  • Glucocorticoids / therapeutic use
  • Humans
  • Immune Complex Diseases / complications*
  • Immune Complex Diseases / diagnosis
  • Immune Complex Diseases / drug therapy
  • Immune Complex Diseases / immunology
  • Immunosuppressive Agents / therapeutic use
  • Kidney / drug effects
  • Kidney / immunology*
  • Kidney / pathology
  • Recurrence
  • Time Factors
  • Treatment Outcome
  • Young Adult

Substances

  • Autoantibodies
  • Biomarkers
  • Glucocorticoids
  • Immunosuppressive Agents
  • antiglomerular basement membrane antibody

Supplementary concepts

  • Segmental glomerulosclerosis