Recurrent, Atypical Anti-Glomerular Basement Membrane Disease

Area of Science:

• Nephrology
• Immunology
• Pathology

Background:

• Anti-glomerular basement membrane (GBM) disease, or Goodpasture's disease, typically involves acute kidney injury and lung hemorrhage.
• While usually monophasic, atypical presentations are increasingly noted.

Observation:

• A patient presented with lung hemorrhage and minimal renal involvement, but negative serum anti-GBM antibodies.
• Renal biopsy immunofluorescence confirmed the diagnosis, leading to effective treatment.
• Remarkably, the patient had a similar episode 10 years prior with positive anti-GBM antibodies and more severe kidney disease.

Findings:

• This case demonstrates a rare presentation of anti-GBM disease with negative serology and non-proliferative, non-crescentic glomerulonephritis.
• It also highlights the possibility of disease recurrence over a decade.
• Renal biopsy immunofluorescence proved crucial for diagnosis when serum antibodies were undetectable.

Implications:

• Increased awareness of variable anti-GBM disease presentations, including negative serology and indolent forms, is necessary.
• The findings underscore the diagnostic value of renal biopsy in suspected anti-GBM disease, even with negative serum tests.
• This case contributes to understanding the potential for late recurrence and atypical histology in anti-GBM nephritis.