Rapid progressive glomerulonephritis (RPGN) (synonyms: Glomerulonephritis, rapidly progressive; English rapidly progressive glomerulonephritis ICD-10 N01.-: Rapid-progressive nephritic syndrome) is a relatively rare form of glomerulonephritis with rapidly progressive (progressive) deterioration of kidney function.
Glomerulonephritis is bilateral inflammation of the kidneys in which the renal corpuscles (glomerules) are affected first.
It is a nephrological emergency that must be clarified and treated, otherwise renal insufficiency may develop (within days to weeks).
The following main forms of glomerulonephritis are distinguished:
- Minimal-change glomerulonephritis (MCGN) (glomerular minimal lesion) – most common cause of nephrotic syndrome in childhood.
- Focal segmental sclerosing glomerulonephritis (FSSGN) – associated with nephrotic syndrome in approximately 15% of cases.
- Membranous glomerulonephritis (MGN) – the most common cause of nephrotic syndrome in adults; accounts for 20-30% of all glomerulonephritides; may be primary or secondary (resulting from other diseases)
- Membranoproliferative glomerulonephritis (MPGN) – associated with nephrotic syndrome in 50%.
- Mesangial IgA glomerulonephritis – most common form with up to 35% of cases.
- Rapid progressive glomerulonephritis (RPGN) – occurs in 2-7% of patients; the disease is classified into several causative groups
Rapid progressive glomerulonephritis (RPGN) is divided into the following types:
- Type 1: 12% of cases; due to antibodies against the glomerular basement membrane.
- Type 2: 44% of cases; caused by deposition of immune complexes; occurs, for example, in the presence of lupus erythematosus (LE)
- Type 3: 44% of cases; immune complexes and antibasal membrane antibodies are absent.
The incidence (frequency of new cases) is approximately 1 disease per 100,000 inhabitants per year (in Germany).
Course and prognosis: If left untreated, the rapid (rapidly) progressive glomerulonephritis leads to terminal renal failure (kidney failure). If therapy is started early, i.e., the kidneys still have residual function, renal function improves in more than 60% of cases.
Types 2 and 3 of rapid progressive glomerulonephritis are often recurrent (recurring).
