Abstract
Rapidly progressive glomerulonephritis (RPGN) is defined clinically by the rapid decrease of renal function and histologically as crescentic glomerulonephritis with more than 50% crescents. The most common cause of RPGN is ANCA-associated vasculitis while anti-glomerular basement membrane (anti-GBM) disease and RPGN caused by immune complex diseases (e.g. systemic lupus erythematosus) are relatively rare.
Early establishment of the right diagnosis and immediate treatment are crucial for patients' prognosis. First choice therapy includes high-dose corticosteroids and cyclophosphamide, with plasma exchange (or immunoadsorption) added in some cases.
Newer therapeutic possibilities include especially rituximab even though there is limited experience with its use in the settings of more severe RPGN or pulmo-renal syndrome. Results of currently ongoing and future trials should help to establish the exact role of plasma exchange in the therapy of RPGN, and prove efficacy and safety of novel therapeutic options.