American Board of Internal Medicine (ABIM) Certification Practice Exam

What disease is characterized by RPGN, particularly in Goodpasture syndrome?

1. Diadic Glomerulonephropathy

2. Alport Syndrome

3. Granulomatosis with Polyangiitis

4. Membranous Nephropathy

The correct answer is: Granulomatosis with Polyangiitis

Goodpasture syndrome is an autoimmune condition characterized by the presence of anti-glomerular basement membrane antibodies, which specifically target the kidneys and lungs. This often leads to rapidly progressive glomerulonephritis (RPGN) and pulmonary hemorrhage. The key feature of RPGN in this context is the acute onset of kidney failure, typically accompanied by significant proteinuria, hematuria, and a decline in renal function. Granulomatosis with Polyangiitis, also known as Wegener's granulomatosis, is an autoimmune vasculitis that affects multiple organ systems and is characterized by glomerulonephritis, pulmonary manifestations, and systemic symptoms. In this condition, RPGN can also occur, and while it is not the same mechanism as in Goodpasture syndrome, it can present with similar acute renal failure due to the destruction of glomeruli. The relationship to RPGN in Goodpasture syndrome arises from its classification within the broader spectrum of conditions that cause RPGN, with anti-GBM disease being one of the classic forms. This makes Granulomatosis with Polyangiitis closely associated given its presentations of RPGN as well, although it has a different pathophysiology. In contrast