Renal-limited vasculitis (RLV)

Abstract

This chapter uses the term renal-limited vasculitis (RLV) to refer to disorders of widely different aetiology and pathogenesis having in common the development of destructive lesions of the glomerular capillaries often leading to segmental necrosis of the capillary wall and proliferation of cells within Bowman’s space (crescents) in the absence of any multi-system manifestations. The resulting accumulation of cells gives rise to a ‘crescent’ enveloping the glomerular tuft itself; polymerization of fibrinogen in Bowman’s space due to passage of fibrinogen through gaps in the damaged capillary wall, the elaboration of procoagulant factors by infiltrating monocytes, and impaired fibrinolysis all contribute to its pathogenesis. Quite often the crescentic lesions are extensive and involve a majority of glomeruli. Such patients frequently manifest rapid and progressive deterioration of renal function leading to the clinical syndrome of rapidly progressive glomerulonephritis. This chapter discusses the pathology, prognosis,,treatment options, and risk factors for RLV.