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Introduction to Renal Aspects of Vasculitis SAP

1. The systemic vasculitides that affect the kidney are primarily those that target medium- and small-arteries. t 2. The systemic vasculitides can be classified on the basis of: 1) the size of the arteries they affect; 2) demographic high-risk subgroups; 3) predilection for various organs; 4) concomitant granulomatous inflammation; and 5) presence of specific antibodies in the patients serum. t 3. Antineutrophil cytoplasmic antibodies directed against protease 3 (PR3) are more commonly associated with Wegeners granulomatosis, whereas those directed against myeloperoxidase (MPO) are more commonly seen with microscopic polyangiitis or Churg-Strauss syndrome. t 4. Although the classic presentation of the patient with periarteritis nodosa involves postprandial abdominal pain, the most common renal manifestation is arteritis of the small- and medium-sized intrarenal arteries, which causes renin release from the juxtaglomerular apparatus, hypertension, and infarction of segments of the kidney. t 5. Cyclophosphamide, an alkylating agent, is often useful for patients with periarteritis nodosa that do not respond to corticosteroids, or who have arteritic ischemia of major organs. t 6. The typical renal manifestation of Wegeners granulomatosis is rapidly progressive glomerulonephritis with non-nephrotic range proteinuria, and, if left untreated, often leads to end-stage renal disease within days to weeks. t 7. Despite much research, the prognosis of patients with Wegeners granulomatosis remains so dismal that it is still called fatal midline granuloma in most parts of the world.f 8. Microscopic polyangiitis and Wegeners granulomatosis are somewhat similar, but can be distinguished by granuloma formation, different subtypes of ANCA, and severity of upper respiratory tract (especially nasal) symptoms. t 9. Most patients with Churg-Strauss syndrome present with asthma and eosinophilia, and develop features of vasculitis, which can affect any organ (including the kidney), in the later stages of their disease. t 10.Vasculitis associated with mixed cryoglobulinemia (because these cryoglobulins consist of both IgG and IgM antibodies) is commonly associated with chronic hepatitis C infection. t