title: Treatment of severe renal disease in ANCA positive and negative small vessel vasculitis with rituximab.
abstract: OBJECTIVE
  Rituximab and glucocorticoids are a non-inferior alternative to cyclophosphamide and glucocorticoid therapy for induction of remission in antineutrophil cytoplasmic antibody (ANCA) associated vasculitis (AAV) patients 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 remission in patients with AAV and ANCA-negative vasculitis presenting 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 induction with or without plasmapheresis were included. Fourteen patients met the inclusion criteria. The primary outcomes were rate of remission and dialysis independence at 6 months. The secondary outcomes were eGFR at 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 ultimately developed ESRD, and one died during the follow-up period.
  
  
  CONCLUSIONS
  Patients with AAV and severe renal disease achieve high rates of remission and dialysis independence when treated with rituximab and glucocorticoids without cyclophosphamide.
authors.affiliation: Department of Medicine, Johns Hopkins University, Baltimore, Md., USA.
authors.forename: Shivani
authors.initials: S
authors.lastname: Shah
authors.forename: Zdenka
authors.initials: Z
authors.lastname: Hruskova
authors.forename: Marten
authors.initials: M
authors.lastname: Segelmark
authors.forename: Matthew D
authors.initials: MD
authors.lastname: Morgan
authors.forename: Jonathan
authors.initials: J
authors.lastname: Hogan
authors.forename: Steven K
authors.initials: SK
authors.lastname: Lee
authors.forename: Jessica
authors.initials: J
authors.lastname: Dale
authors.forename: Lorraine
authors.initials: L
authors.lastname: Harper
authors.forename: Vladimir
authors.initials: V
authors.lastname: Tesar
authors.forename: David R W
authors.initials: DR
authors.lastname: Jayne
authors.forename: Duvuru
authors.initials: D
authors.lastname: Geetha
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000276:immunology / Q000628:therapy; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D015331:Cohort Studies; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use*; D014890:Granulomatosis with Polyangiitis / Q000276:immunology / Q000628:therapy*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D007676:Kidney Failure, Chronic; D008297:Male; D055953:Microscopic Polyangiitis / Q000276:immunology / Q000628:therapy*; D008875:Middle Aged; D053146:Myeloblastin / Q000276:immunology; D009195:Peroxidase / Q000276:immunology; D010956:Plasmapheresis; D012074:Remission Induction; D051436:Renal Insufficiency, Chronic / Q000276:immunology / Q000628:therapy*; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use*; D012720:Severity of Illness Index; D016896:Treatment Outcome; D014657:Vasculitis / Q000276:immunology / Q000628:therapy
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007155:Immunologic Factors; D000069283:Rituximab; D009195:Peroxidase; D053146:Myeloblastin