The clinical spectrum of primary renal vasculitis.

Background And Objectives: The vasculitides are potentially severe and often difficult to diagnose syndromes. Many forms of vasculitis may involve the kidneys. This review will focus on the clinical and histopathological aspects of renal involvement in the systemic vasculitides.

Methods: We searched the MEDLINE database using as key terms the MeSH terms and textwords for different forms of vasculitis and for renal involvement, creating a database of more than 2200 relevant references.

Results: The frequency of renal involvement in vasculitis varies among different syndromes. It is more frequent in Wegener's granulomatosis and microscopic polyarteritis, while it is uncommon to rare in other forms of vasculitis such as Behçet's disease and relapsing polychondritis. The vessels affected include the renal artery in Takayasu arteritis, medium-size renal parenchymal artery in classic polyarteritis nodosa, and glomerular involvement in Wegener's granulomatosis and microscopic polyarteritis. The clinical expression of renal vasculitis depends on the size of the affected vessels and includes renovascular hypertension, isolated nonnephrotic proteinuria, interstitial nephritis, and glomerulonephritis, which can be rapidly progressive. Diagnosis is established by a combination of history, clinical manifestations, laboratory findings (eg, urine sediment, urine protein, antineutrophil cytoplasmic antibodies), imaging techniques (renal angiography, especially when there is a suspicion of medium-to-large vessel disease, and chest radiograph), and finally, renal biopsy. Prognosis varies from unfavorable in the rapidly progressive glomerulonephritis of microscopic polyarteritis, which can lead to renal failure, chronic dialysis, and renal transplantation, to benign, as in the case of Henoch Schonlein purpura, in which the majority of patients recover.

Conclusions: The manifestations and prognosis of renal vasculitis range widely. Renal involvement greatly influences prognosis and dictates the need for early and prompt immunosuppressive therapy. Thus, the clinician should be alert for the timely diagnosis and treatment of renal vasculitis.