Treatment of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

An Unusual Case of Hematuria

“…Daily oral CYC plus corticosteroids remains the gold standard therapy for this group of patients (28). Studies have found remission rates between 70% and 100% and early mortality rates of Ͻ20% with increased treatment-related morbidity (28). Our patient presented with a serum creatinine level Ͻ500 moles/liter (3.8 mg/dl) and based on these recommendations was treated with prednisone and oral CYC (30,31).…”

“…Randomized controlled trials have been conducted in order to obtain evidence-based recommendations (28 -30). Daily oral CYC plus corticosteroids remains the gold standard therapy for this group of patients (28). Studies have found remission rates between 70% and 100% and early mortality rates of Ͻ20% with increased treatment-related morbidity (28).…”

“…Once the disease is in remission (usually within 3-6 months), azathioprine is as effective as CYC and more effective than mycophenolate mofetil to maintain remission (28,29,31). Bosch et al reported that azathioprine and daily prednisone can maintain remission in patients who test negative for ANCAs, whereas patients positive for PR3 ANCA should be closely monitored because of a higher probability of relapse (28).…”

“…Bosch et al reported that azathioprine and daily prednisone can maintain remission in patients who test negative for ANCAs, whereas patients positive for PR3 ANCA should be closely monitored because of a higher probability of relapse (28). Methotrexate has been shown to be equivalent to azathioprine, but it should not be used in patients with renal impairment (45).…”

“…Substantial in vitro and in vivo evidence suggests that ANCAs have an important role in the pathogenesis of vasculitis in AAV, and are not simply a serologic marker of the disease (28). Neutrophils are likely to have a major role in ANCAnegative pauci-immune crescentic glomerulonephritis (8,46).…”