Atypical form of Goodpasture's disease

Vnitr Lek. 2023 Fall;69(5):316-321. doi: 10.36290/vnl.2023.062.

Abstract

Goodpasture's disease and anti-glomerular basement membrane nephritis (anti-GBM nephritis) are rare autoimmune small vessel vasculitis predominantly affecting young men. Goodpasture's disease plays an important part in differential diagnosis of pulmonary - renal syndrome. The evidence of circulating autoantibodies, a typical histological appearance of the kidney biopsy with finding of the crescent glomerulonephritis and clinical presentation of nephritic syndrome play an important role in diagnosis. Our case report describes a case of a young male with anti-GBM nephritis that presents as rapidly progressive glomerulonephritis (RPGN) with progression to dialysis-dependent renal failure. The atypical sign of the case was development of nephrotic syndrome with volume-dependent hypertension. The case was complicated by heparin-induced thrombocytopenia. During combined immunosuppressive therapy with plasmapheresis, the condition of the patient has stabilized. However, renal function did not recover. This previously fatal disease has nowadays a very good survival rate because of immunosuppresion therapy, plasmapheresis and hemodialysis.

Keywords: anti-GBM nephritis; immunosuppression; Goodpasture’s disease; immunosuppression; nephritic syndrome; plasma-exchange; plasmapheresis; rapidly progressive glomerulonephritis.

Publication types

  • Case Reports

MeSH terms

  • Anti-Glomerular Basement Membrane Disease* / diagnosis
  • Anti-Glomerular Basement Membrane Disease* / therapy
  • Autoantibodies / therapeutic use
  • Glomerulonephritis* / complications
  • Glomerulonephritis* / pathology
  • Hemorrhage / etiology
  • Humans
  • Male
  • Nephritis* / complications

Substances

  • Autoantibodies

Supplementary concepts

  • Rapidly progressive glomerulonephritis with pulmonary hemorrhage