E-Mail karger@karger.com Original Report: Patient-Oriented, Translational Research Am J Nephrol 2015;41:296–301 DOI: 10.1159/000431336 Treatment of Severe Renal Disease in ANCA Positive and Negative Small Vessel Vasculitis with Rituximab Shivani Shah   a Zdenka Hruskova   c Marten Segelmark   d Matthew D. Morgan   e Jonathan Hogan   b Steven K. Lee   a Jessica Dale   f Lorraine Harper   e Vladimir Tesar   c David R.W. Jayne   g Duvuru Geetha   a   a  Department of Medicine, Johns Hopkins University, Baltimore, Md., b  Department of Medicine, Hospital of the University of Pennsylvania, Philadelphia, Pa., USA; c  Department of Nephrology, Charles University, Prague, Czech Republic; d  Department of Medicine, Linköping University, Linköping, Sweden; e  University of Birmingham and f  School of Immunity and Infection, College of Medical and Dental Sciences, University of Birmingham, Birmingham, g  Vasculitis and Lupus Clinic, Addenbrooke’s Hospital, Cambridge, UK 6 months, end-stage renal disease (ESRD), survival rates and adverse events. Results: All patients were Caucasian, and 57% were male. The mean eGFR was 12 ml/min/1.73 m 2 at diagnosis. All patients achieved remission with a median time to remission of 55 days. Seven patients required dialysis at presentation of which 5 patients recovered renal function and discontinued dialysis by 6-month follow-up. The mean eGFR for the 11 patients without ESRD who completed 6-month follow-up was 33 ml/min/1.73 m 2 . Four patients ul- timately developed ESRD, and one died during the follow-up period. Conclusion: Patients with AAV and severe renal dis- ease achieve high rates of remission and dialysis indepen- dence when treated with rituximab and glucocorticoids without cyclophosphamide. © 2015 S. Karger AG, Basel Introduction Antineutrophil cytoplasmic antibody (ANCA) associ- ated vasculitis (AAV) is the most common cause of rap- idly progressive glomerulonephritis. Approximately, 75– 90% of patients with ANCA vasculitis develop renal in- Key Words Rituximab · ANCA vasculitis · Severe renal disease Abstract Background/Aims: Rituximab and glucocorticoids are a non-inferior alternative to cyclophosphamide and glucocor- ticoid therapy for induction of remission in antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) pa- tients with moderate renal disease. The efficacy and safety of this approach in patients with severe renal impairment are unknown. We report the outcomes and safety profile of rituximab and glucocorticoid therapy for induction of remis- sion in patients with AAV and ANCA-negative vasculitis pre- senting with severe renal disease. Methods: A multicenter, retrospective, cohort study was conducted between 2005 and 2014. Patients with new or relapsing disease with an estimated glomerular filtration rate (eGFR) of ≤20 ml/ min/1.73 m 2 treated with rituximab and glucocorticoid in- duction with or without plasmapheresis were included. Fourteen patients met the inclusion criteria. The primary outcomes were rate of remission and dialysis indepen- dence at 6 months. The secondary outcomes were eGFR at Received: March 12, 2015 Accepted: May 6, 2015 Published online: June 2, 2015 Nephrolo gy American Journal of Duvuru Geetha, MD, MRCP (UK) Department of Medicine Johns Hopkins University School of Medicine Baltimore, MD 21224 (USA) E-Mail gduvura  @  jhmi.edu © 2015 S. Karger AG, Basel 0250–8095/15/0415–0296$39.50/0 www.karger.com/ajn