Long-term Clinical Course of Antineutrophil Cytoplasmic Antibody-associated Vasculitis Patients off Maintenance Therapy.

Objectives The optimal duration of maintenance immunosuppressive therapy in patients with antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is still controversial. The aim of our study is to describe the characteristics and outcomes of patients with AAV who were able to stop maintenance agents completely while remaining on daily prednisone (< 5 mg) for at least 36 months. Materials and methods AAV patients treated at our center from 2000 to 2016 and who were not on maintenance agents while remaining on prednisone < 5 mg daily for at least 36 months were identified by the providers, and their records were retrospectively reviewed. Relapse was defined by the reinitiation of immunosuppressive therapy for biopsy-proven glomerulonephritis or any extra-renal organ involvement. Results Of the 18 patients who fulfilled the study inclusion criteria, 12 were male and 14 were Caucasian. The mean age at AAV diagnosis was 54 years. Seventeen patients had renal involvement and seven had lung involvement. Eleven patients received cyclophosphamide and eight patients received rituximab along with glucocorticoids for remission induction. Twelve patients were weaned completely off prednisone. The median duration of prednisone use was 20 months. Nine patients received maintenance therapy with azathioprine or mycophenolate mofetil. The median duration of maintenance therapy was 24 months. The mean follow-up time after stopping the maintenance agent was 64 months. During this period, three patients had disease relapse. Conclusions Stopping maintenance agents for > 36 months can be achieved in some patients with AAV. Prospective, randomized controlled trials are needed to confirm this finding.