A Randomized Trial of Maintenance Therapy for Vasculitis Associated with Antineutrophil Cytoplasmic Autoantibodies

• Published in: The New England journal of medicine Vol. 349; no. 1; pp. 36 - 44
• Main Authors: Jayne, David, Rasmussen, Niels, Andrassy, Konrad, Bacon, Paul, Tervaert, Jan Willem Cohen, Dadoniené, Jolanta, Ekstrand, Agneta, Gaskin, Gill, Gregorini, Gina, de Groot, Kirsten, Gross, Wolfgang, Hagen, E. Christiaan, Mirapeix, Eduardo, Pettersson, Erna, Siegert, Carl, Sinico, Alberto, Tesar, Vladimir, Westman, Kerstin, Pusey, Charles
• Format: Journal Article
• Language: English
• Published: Boston, MA Massachusetts Medical Society 03.07.2003
• Subjects: Adult; Aged; Antibodies, Antineutrophil Cytoplasmic - blood; Azathioprine - adverse effects; Azathioprine - therapeutic use; Biological and medical sciences; Cardiovascular system; Clinical Medicine; Cyclophosphamide - adverse effects; Cyclophosphamide - therapeutic use; Drug dosages; Drug therapy; Drug Therapy, Combination; Female; Glucocorticoids - therapeutic use; Hepatitis; Humans; Immunosuppressive Agents - adverse effects; Immunosuppressive Agents - therapeutic use; Klinisk medicin; Male; Medical and Health Sciences; Medical sciences; Medicin och hälsovetenskap; Middle Aged; Neutropenia - chemically induced; Pharmacology. Drug treatments; Prednisolone - therapeutic use; Recurrence; Remission Induction; Urologi och njurmedicin; Urology and Nephrology; Vascular wall; Vasculitis - drug therapy; Vasculitis - immunology; Vasculitis - mortality; Human; Statistical analysis; Antineutrophil cytoplasmic antibody; Weaning; Cardiovascular disease; Vascular disease; Chemotherapy; Cyclophosphamide; Oxazaphosphinane derivatives; Randomization; Vasculitis; Cohort study; Nitrogen mustard; Maintenance treatment; Azathioprine
• ISSN: 0028-4793; 1533-4406
• DOI: 10.1056/NEJMoa020286

This randomized trial involved 144 patients who had generalized vasculitis associated with circulating autoantibodies to neutrophil cytoplasmic antigens (ANCA) that was in remission after initial treatment with cyclophosphamide. The rate of relapse was similar among patients receiving maintenance immunosuppressive treatment with azathioprine and among those receiving maintenance treatment with cyclophosphamide. The most common primary systemic vasculitis syndromes — Wegener's granulomatosis, microscopic polyangiitis, and vasculitis limited to the kidneys — are associated with circulating autoantibodies to neutrophil cytoplasmic antigens (ANCA). 1 – 3 It has been suggested that they be grouped together as ANCA-associated vasculitis because of their histologic similarities, the absence of immune deposits in all of them, the potential contribution of ANCA to their pathogenesis, and their similar responses to immunosuppressive therapy. 2 , 4 – 12 Renal involvement is common and is typically manifested as rapidly progressive glomerulonephritis; it results in either death or end-stage renal failure within two years in more than . . .