title: Granulomatosis with polyangiitis (Wegener's): impact of maintenance therapy duration.
abstract: To determine outcomes in relation to duration of maintenance therapy in patients with granulomatosis with polyangiitis (Wegener's) (GPA), we conducted a retrospective chart review of patients with GPA seen at a single vasculitis center from 1992 to 2010. All patients achieved remission defined by a Birmingham Vasculitis Activity Score for Wegener Granulomatosis (BVAS/WG) of 0 with either cyclophosphamide or methotrexate. After achieving remission all patients were started on maintenance therapy with either methotrexate or azathioprine.The study comprised 157 patients with a median follow-up of 3.1 years. Using a univariate model, the continuation of maintenance medications for >18 months showed a 29% reduction in hazard ratio (HR) for relapse (HR, 0.71; 95% confidence interval [CI], 0.42-1.19; p = 0.19). Treatment for >36 months showed a 66% reduction in hazard ratio for relapse (HR, 0.34; 95% CI, 0.15-0.76; p = 0.008). When length of treatment was considered as a continuous factor, longer courses had an inverse relationship with the risk of relapse (HR, 0.70; 95% CI, 0.58-0.84; p < 0.001), which remained significant after adjusting for prednisone dose (HR, 0.59; 95% CI, 0.42-0.83; p = 0.003). Fifty-two percent of relapses occurred while the patients were off maintenance therapy. Among all patients who relapsed on therapy, 52% of those receiving methotrexate were on <15 mg/week, and 67% of those receiving azathioprine were on ≤ 50 mg/d. There were no differences between the short- and long-term maintenance therapy groups in overall adverse events or GPA-related morbidity.Discontinuation or use of low doses of maintenance therapy is associated with a higher relapse rate.
authors.affiliation: From Department of Rheumatic and Immunologic Diseases (JS); Department of Quantitative Health Sciences (BN); and Center for Vasculitis Care and Research, Department of Rheumatic and Immunologic Diseases (CAL, GSH, AVF), Cleveland Clinic, Cleveland, Ohio. Current affiliation: University of Kansas Medical Center, Kansas City, Kansas.
authors.forename: Jason
authors.initials: J
authors.lastname: Springer
authors.forename: Benjamin
authors.initials: B
authors.lastname: Nutter
authors.forename: Carol A
authors.initials: CA
authors.lastname: Langford
authors.forename: Gary S
authors.initials: GS
authors.lastname: Hoffman
authors.forename: Alexandra
authors.initials: A
authors.lastname: Villa-Forte
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D001379:Azathioprine / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D002648:Child; D004334:Drug Administration Schedule; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D008297:Male; D008727:Methotrexate / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D008875:Middle Aged; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D013997:Time Factors; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D001379:Azathioprine; D008727:Methotrexate