Lack of Superiority of Steroids Plus Plasma Exchange to Steroids Alone in the Treatment of Polyarteritis Nodosa and Churg‐Strauss Syndrome

Abstract

Objective. To define the most effective treatment for polyarteritis nodosa (PAN) and Churg‐Strauss syndrome (CSS). Methods. We conducted a prospective, randomized, multicenter trial in which 78 patients were randomly assigned to receive either prednisone and plasma exchange (group A; n = 36) or prednisone alone (group B; n = 42) as first‐line treatment of PAN and CSS. Patients with hepatitis B virus—related PAN were not included in this study. The end point of the study was control of the disease (recovery and remission) or death. Results. Clinical symptoms and laboratory findings did not differ statistically in the 2 groups at study entry. Initial control of the disease was similar in both groups. The assigned treatment was stopped in 16 patients because of lack of efficacy. Oral cyclophosphamide or dapsone therapy reversed the disease evolution in 7 of these 10 group A patients and in 4 of these 6 group B patients. At 7 years of followup, 56 patients had completely recovered (27 in group A, 29 in group B), 7 patients were in clinical remission, and 15 patients had died (19.2%; 6 group A patients and 9 group B patients). The prednisone—plasma exchange combination was no more beneficial than corticosteroids alone in preventing relapses over the long term. There was no significant difference in the 7‐year cumulative survival rates of the two groups (83% and 79%, respectively). Conclusion. Based on our data, we conclude that combined treatment with prednisone and plasma exchange is not superior to treatment with prednisone alone and must not be systematically employed for initial treatment of PAN and CSS. In most cases, cyclophosphamide as second‐line treatment is effective and well tolerated.

Microscopic polyangiitis: Clinical and laboratory findings in eighty-five patients

“…Patients were selected from the database of the French Vasculitis Study Group, which was developed over the last 15 years from several multicenter therapeutic trials on PAN, Churg-Strauss syndrome (4)(5)(6)(7)(8), and Wegener's granulomatosis (9). Clinical and laboratory data had been collected prospectively.…”

“…Seventy-one patients were enrolled in prospective therapeutic trials organized by the French Vasculitis Study Group; the results have been published elsewhere (4)(5)(6)(7)(8)(9). These 71 patients received the treatments being administered in the trials at the time of inclusion.…”

Treatment of Antineutrophil Cytoplasmic Antibody–Associated Vasculitis

“…Most studies of Churg-Strauss syndrome treatment have been performed by the French Vasculitis Study Group, [91][92][93][94][95] which proposed the 5-factors score as a predictor of death in Churg-Strauss syndrome 96 : (1) renal insufficiency (creatinine level Ͼ1.58 mg/ dL); (2) proteinuria higher than 1 g/d; (3) gastrointestinal bleeding, perforation, infarction, or pancreatitis; (4) central nervous system involvement; and (5) cardiomyopathy. The presence of each factor is given 1 point.…”

S1 guidelines Diagnostics and treatment of ANCA-associated vasculitis

“…Bei der EGPA scheint die zur Standardinduktionstherapie zusätzlich durchgeführte Plasmaseparation nicht wirksam zu sein [62][63][64]. Jedoch ist die schwergradige RPGN, die bei GPA und MPA eine mögliche Indikation zur Plasmaseparation darstellt, bei der EGPA nicht gezielt untersucht worden.…”