CORTICOSTEROIDS PLUS PULSE CYCLOPHOSPHAMIDE AND PLASMA EXCHANGES VERSUS CORTICOSTEROIDS PLUS PULSE CYCLOPHOSPHAMIDE ALONE IN THE TREATMENT OF POLYARTERITIS-NODOSA AND CHURG-STRAUSS-SYNDROME PATIENTS WITH FACTORS PREDICTING POOR-PROGNOSIS - A PROSPECTIVE, RANDOMIZED TRIAL IN 62 PATIENTS

Objective. To define the most effective treatment for severe polyarter itis nodosa (PAN) and Churg-Strauss syndrome (CSS) and to investigate the indication for plasma exchange treatment. Methods. We conducted a prospective, randomized, multicenter trial in which 62 patients were r andomly assigned to receive either prednisone plus cyclophosphamide (i ntravenous bolus) (group A; n = 28) or prednisone plus cyclophosphamid e (intravenous bolus) plus plasma exchanges (group B; n = 34) as first -line treatment for severe PAN or CSS. Factors predicting poor prognos is were renal symptoms, gastrointestinal tract involvement, cardiomyop athy, central nervous system involvement, weight loss >10% of body wei ght, and age >50 years old. Patients with hepatitis B virus-related PA N were not included in this study. The end point of the study was cont rol of the disease (recovery or remission) or death. Results. Clinical symptoms and laboratory findings did not differ significantly in the 2 groups. Initial control of the disease was similar in both groups. R elapse after initial control of the disease was observed in 7 patients (4 in group A and 3 in group B). The mean +/- SD followup period was 31.1 +/- 20 months for group A and 35.9 +/- 16.8 months for group B. A t 5 years of followup, 38 patients (61.3%) were cured (16 in group A a nd 22 in group B), and 5 (8.1%) were in remission without treatment bu t had not yet completed the cure-defining period of 18 months (3 in gr oup A and 2 in group B), Eight (12.9%) (2 in group A and 6 in group B) were considered to be in clinical remission and required a maintenanc e regimen of low-dose corticosteroids. Eleven patients died during the study period (7 in group A [25%], 4 in group B [11.8%]). Uncontrolled vasculitis was responsible for 4 deaths (2 in each group), and treatm ent side effects caused the death of 1 patient in group A. There was n o significant difference between the 5-year cumulative survival rates of the 2 groups (75% and 88%, respectively). Conclusion. Based on our data, combined treatment with prednisone, cyclophosphamide, and plasma exchanges is not superior to treatment with prednisone and cyclophoph amide alone, and plasma exchanges should not be systematically propose d for initial treatment of severe PAN or CSS.