What is the syndrome of rapidly progressive glomerulonephritis (RPGN)?
Patients with Syndrome of rapidly progressive glomerulonephritis have evidence of glomerular disease (proteinuria, hematuria, and red cell casts) accompanied by rapid loss of kidney function over days to weeks. If untreated, RPGN often results in kidney failure.
The pathologic hallmark of Syndrome of rapidly progressive glomerulonephritis is the presence of crescents on kidney biopsy, and RPGN is also described as crescentic nephritis (see later for further discussion). Fortunately, the disorders associated with this syndrome are rare, so that RPGN makes up only 2% to 4% of all cases of glomerulonephritis.
Importantly, Syndrome of rapidly progressive glomerulonephritis is not a specific diagnosis, and multiple different diseases can cause this syndrome.
Diagnosis almost always requires a biopsy of the affected tissue if the presentation suggests systemic involvement, or of the kidney if it is kidney-limited.
