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Overview of the classification and treatment of rapidly progressive (crescentic) glomerulonephritis

INTRODUCTION

Rapidly progressive glomerulonephritis (RPGN) is a clinical syndrome manifested by features of glomerular disease in the urine and by progressive loss of renal function over a comparatively short period of time (days, weeks or months). It is characterized morphologically by extensive crescent formation [1]. This histologic finding is a common finding in the elderly presenting with acute nephritis [2].

The severity of the disease is in part related to the degree of crescent formation: patients with circumferential crescents in more than 80 percent of the glomeruli tends to present with advanced renal failure that may not respond well to therapy (picture 1A-E). By comparison, patients with crescents in less than 50 percent of the glomeruli, particularly if the crescents are noncircumferential, typically follow a more indolent course or in rare cases even undergo a remission [3].

PATHOGENESIS OF CRESCENT FORMATION

Crescent formation appears to represent a nonspecific response to severe injury to the glomerular capillary wall [4]. Rents are induced in the glomerular capillary wall, resulting in the movement of plasma products, including fibrinogen, into Bowman's space with subsequent fibrin formation, the influx of macrophages and T cells, and the release of proinflammatory cytokines, such as interleukin-1 and tumor necrosis factor-alpha [5,6]. Thus, crescents may be seen with any form of severe glomerular disease, including lupus nephritis and postinfectious glomerulonephritis, disorders that can usually be diagnosed from the history and typical immunofluorescence and electron microscopic findings. (See "Mechanisms of glomerular crescent formation".)

The stage of active inflammation is often followed by the development of fibrocellular and fibrous crescents [5]. Collagen deposition is due to fibroblast proliferation that is driven by fibroblast growth factors. Transforming growth factor-beta is also thought to play an important role. This transition is important clinically because fibrous crescents represent a stage of the disease that is not likely to respond to immunosuppressive therapy.

TYPES OF CRESCENTIC GN

The term RPGN refers to crescentic glomerulonephritis that is usually due to one of four disorders, which reflect different mechanisms of glomerular injury [1]:
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