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The kidney in systemic vasculitis 

The kidney in systemic vasculitis
The kidney in systemic vasculitis

David Jayne

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date: 25 February 2021

Systemic vasculitis can occur as a primary autoimmune disorder, or as a secondary manifestation of another disease process (related to infection, malignancy, chronic inflammatory disorder, or drugs). Primary systemic vasculitis is classified according to the predominant size of the blood vessel involved and the presence of circulating antineutrophil cytoplasm autoantibodies (ANCA). Incidence and prevalence rates are between 15 and 20 per million and 200 to 400 per million population, respectively. Vasculitic syndromes frequently involve the kidney, causing tissue infarction, loss of function, and rapid progression to endstage renal disease within weeks or months. They account for 5% of cases of endstage renal failure. ANCA-associated vasculitis is the most common cause of renal vasculitis and has been the focus for most research. Management aims for an early diagnosis, recovery of renal function, and prevention of renal relapse. Small-vessel vasculitides—renal disease is common. There are two subgroups: ANCA-associated vasculitis that comprises three syndromes: granulomatosis with polyangiitis (formerly Wegener’s granulomatosis), microscopic polyangiitis, and eosinophilic granulomatosis with angiitis (formerly Churg–Strauss syndrome). The second subgroup is immune complex vasculitis and comprises IgA vasculitis (formerly Henoch–Schönlein purpura), antiglomerular basement membrane disease, and cryoglobulinaemia: these are ANCA negative and characterized by immune complex deposition. Medium- and larger-vessel vasculitides—renal disease is uncommon in the medium-vessel disorders polyarteritis nodosa (ANCA negative) and Kawasaki’s disease, and rare in the large-vessel disorders, giant cell arteritis and Takayasu’s arteritis. This chapter discusses the aetiology and pathogenesis, pathology, clinical presentation, diagnosis, management, disease relapse, and prognosis of systemic vasculitis in detail.

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