Abstract

Multi-system autoimmune diseases, including systemic lupus erythematosus (SLE) and vasculitis, are inflammatory conditions of unknown cause. Renal involvement can occur in a variety of forms and usually represents a severe disease manifestation. SLE is frequently complicated by renal involvement (lupus nephritis). The main renal manifestation of vasculitis is ‘rapidly progressive glomerulonephritis’ (RPGN). This is a potentially reversible, pathological process of fibrinoid necrosis and crescent formation, which, if left untreated, results in end-stage renal failure and death within days to weeks. RPGN is most frequently seen as a manifestation of systemic vasculitis, but also occurs in SLE. Other less frequent examples of renal involvement in multi-system autoimmune disease include vascular lesions in scleroderma, glomerular lesions in anti-glomerular basement membrane disease and interstitial nephritis or glomerular lesions in Sjögren’s syndrome or rheumatoid arthritis. Lupus nephritis and RPGN complicating systemic vasculitis are the most frequent causes of renal failure in multi-system autoimmunity and are discussed in this contribution.

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