Recurrent, Atypical Anti-Glomerular Basement Membrane Disease

Abstract

Anti-glomerular basement membrane disease (GBM) (Goodpasture's disease) typically presents with acute manifestations of rapidly progressive glomerulonephritis often accompanied by lung haemorrhage. Anti-GBM disease is usually monophasic. However, atypical presentations with indolent renal involvement are being increasingly recognized. Herein we report a patient who presented with lung haemorrhage, minimal renal involvement, and negative result for serum anti-GBM antibody, while immunofluorescence examination of the renal biopsy provided the diagnosis leading to the institution right treatment with excellent response. Interestingly, he had presented 10 years earlier with lung hemorrhage, more significant renal involvement clinically and histologically, with positive serum anti-GBM antibody. The present case is intended to increase our awareness regarding the variable presentations of anti-GBM disease, such as with negative serology and recurrence of anti-GBM disease. The presentation of anti-GBM nephritis with non-proliferative, non-crescentic glomerulonephritis is also highlighted. The possible explanations for negative serum anti-GBM antibody are explored with a brief review of literature.

Keywords: Atypical anti-GBM disease; Goodpasture's disease; lung haemorrhage; rapidly progressive glomerulonephritis; recurrent anti-GBM disease.

Figure 1

CT Chest: Diffuse parenchymal opacities on current presentation

Figure 2

KIDNEY BIOPSY on current presentation: Light Microscopy: The Glomerulus is essentially normal, and shows no proliferative or crescentic glomerulonephritis. (PAS stain ×40) Immunofluorescence: Section shows linear IgG along the glomerular basement membrane. (IgG ×40)