Summarizing the evidence regarding a pathogenic role for ANCA in AASV, it is likely that these antibodies are necessary but not sufficient for the development of the clinical syndromes.
The goals of treatment in AASV are initially induction of response and then maintenance of remission.
Traditionally, AASV, such as Wegener's granulomatosis, had mortality rates in excess of 80% within 3 years without adequate treatment.
This article will review three alternatives for induction of response in patients with AASV, two nontraditional management approaches for the maintenance of disease, the positive results associated with the use of co-trimoxazole for patients with Wegener's granulomatosis, and the negative experience with the use of the biologic agent etanercept.
A trial in 2001 found that the intravenous route was as effective at inducing remission of AASV and was associated with fewer infectious complications and leukopenia, as compared to the oral route, but the intravenous route was associated with the risk of more relapses.
EUVAS also formally studied the role of methotrexate in the treatment of AASV via the NORAM trial (Non-Renal Alternative with methotrexate), which directly compared oral methotrexate (20 to 25 mg/week) plus prednisone to oral cyclophosphamide (2 mg/kg/day) plus prednisone in the management of AASV.
In conclusion, plasma exchanges are preferred over pulse steroids in the initial management of severe AASV with renal insufficiency.
The refractory nature of their AASV was defined as active disease not controlled by maximally tolerated cyclophosphamide and corticosteroids (N=8) or a contraindication to cyclophosphamide manifested as drug-induced bladder toxicity (N=1) or prolonged cytopenias (N=2).