Treatment of polyarteritis nodosa and Churg-Strauss syndrome. A meta-analysis of 3 prospective controlled trials including 182 patients over 12 years.

To define the most effective treatment for polyarteritis nodosa (PAN) and Churg-Strauss syndrome (CSS), we undertook 3 consecutive prospective therapeutic trials including 182 patients and tried to answer several important questions: should cyclophosphamide (CYC) be given as the first-line treatment? what is the place of plasma exchanges (PE) in the treatment of systemic vasculitis? does hepatitis B virus (HBV)-related PAN require specific treatment? Our first randomized trial in 71 patients compared the association of CYC with corticosteroids (CS) and PE to CS and PE, in order to evaluate the efficacy of CYC given as the first-line treatment to control disease activity and subsequent survival of PAN and CSS patients. Between December, 1983, and December, 1988, we conducted two trials simultaneously: one aimed at patients without HBV markers and the second at patients with HBV markers. In 78 patients without HBV markers, we compared prednisone and PE to prednisone alone as the initial therapeutic regimen. In 33 patients with PAN related to HBV, a new therapeutic strategy was applied as an alternative to long-term steroid and immunosuppressive therapy: short-term steroid therapy and PE were used to control the evolution of PAN and anti-viral therapy was administered to suppress the etiological agent of the vasculitis. Twelve years after the beginning of the trials on PAN and CSS patients, we think that the therapeutic strategy should be as follow: in PAN without HBV and CSS: Prednisone in association with CYC improves the control of the disease despite infectious side effects which may be reduced by better CYC dose adaptation. It is also possible that CYC could be more effective in some subgroups of PAN, for instance those with clinical symptoms of poor prognosis. We are presently attempting to optimize the CYC prescription (pulses of CYC) in PAN and CSS in the hope of improving prognosis; in PAN related to HBV: The first-line treatment should be the association of anti-viral agents and PE. This treatment was effective and cured a majority of patients within 2 to 3 months; half of them seroconverted. The length of HBV infection before its diagnosis, delay before initiation of treatment and previous immunosuppressive therapy led to a poor seroconversion rate; the role of PE in the treatment of systemic necrotizing angiitis: PE are obviously useful in PAN related to HBV where immune complex deposition has been demonstrated.(ABSTRACT TRUNCATED AT 400 WORDS)