title: Rituximab for the treatment of eosinophilic granulomatosis with polyangiitis (Churg-Strauss).
abstract: BACKGROUND
  Conventional treatment of eosinophilic granulomatosis with polyangiitis (EGPA) (Churg-Strauss) with glucocorticoids, with or without additional immunosuppressive drugs, is limited by partial efficacy, frequent toxicity and a high relapse rate. Rituximab is a licensed treatment for granulomatosis with polyangiitis and microscopic polyangiitis and is of potential benefit to patients with EGPA.
  
  
  METHODS
  Patients with EGPA who received rituximab as single or repeated courses were identified from four vasculitis centres. Standardised data collection was performed, including disease activity status and adverse events, at the time of initial treatment and after 6 and 12 months. Remission was defined as a Birmingham Vasculitis Activity Score (BVAS) of 0 and partial response as a ≥50% reduction in BVAS compared with baseline.
  
  
  RESULTS
  41 patients (21 women) with EGPA treated with rituximab between 2003 and 2013 were identified. 15 (37%) had refractory, 21 (51%) relapsing and 5 (12%) new onset disease. 19 received a single course and 22 received repeat-dose rituximab to prevent relapse. By 6 months, 83% improved with remission in 34% and partial response in 49%, and by 12 months 49% were in remission and 39% had a partial response. Prednisolone doses decreased in all patients by 6 and 12 months. Antineutrophil cytoplasmic antibody positivity at baseline was associated with a higher remission rate at 12 months. Adverse events included 15 infections (6 were severe).
  
  
  CONCLUSIONS
  The treatment of EGPA with rituximab resulted in high rates of improvement and reduced requirement of prednisolone. Rituximab may be considered for the treatment of EGPA.
authors.affiliation: Department of Renal Medicine, Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK Department of Clinical Sciences, Rheumatology, Lund University, Lund, Sweden.
authors.forename: A J
authors.initials: AJ
authors.lastname: Mohammad
authors.affiliation: Service de Medecine Interne, Hôpital Edouard Herriot, Lyon, France.
authors.forename: A
authors.initials: A
authors.lastname: Hot
authors.affiliation: Klinikum Bad Bramstedt, University Hospital Schleswig-Holstein, Bad Bramstedt, Germany.
authors.forename: F
authors.initials: F
authors.lastname: Arndt
authors.affiliation: Klinikum Bad Bramstedt, University Hospital Schleswig-Holstein, Bad Bramstedt, Germany.
authors.forename: F
authors.initials: F
authors.lastname: Moosig
authors.affiliation: Département universitaire de réanimation et d'urgence médicale, université Lille Nord de, Lille, France.
authors.forename: M-J
authors.initials: MJ
authors.lastname: Guerry
authors.affiliation: Vasculitis Center, Boston Medical Center, Boston University School of Medicine, Boston, Massachusetts, USA.
authors.forename: N
authors.initials: N
authors.lastname: Amudala
authors.affiliation: Department of Renal Medicine, Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK.
authors.forename: R
authors.initials: R
authors.lastname: Smith
authors.affiliation: Department of Respiratory Medicine, Addenbrooke's Hospital, Cambridge, UK.
authors.forename: P
authors.initials: P
authors.lastname: Sivasothy
authors.affiliation: Service de Médecine Interne, Centre de Référence des Maladies Systémiques et Autoimmunes Rares, Université Paris-Descartes, AP-HP, Hôpital Cochin, Paris, France.
authors.forename: L
authors.initials: L
authors.lastname: Guillevin
authors.affiliation: Division of Rheumatology, Vasculitis Center, University of Pennsylvania, Philadelphia, Pennsylvania, USA.
authors.forename: P A
authors.initials: PA
authors.lastname: Merkel
authors.affiliation: Department of Renal Medicine, Vasculitis and Lupus Clinic, Addenbrooke's Hospital, Cambridge, UK.
authors.forename: D R W
authors.initials: DR
authors.lastname: Jayne
mesh_terms: D000328:Adult; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood; D015267:Churg-Strauss Syndrome / Q000097:blood / Q000188:drug therapy*; D004359:Drug Therapy, Combination; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D011239:Prednisolone / Q000627:therapeutic use; D012008:Recurrence; D012074:Remission Induction; D012189:Retrospective Studies; D000069283:Rituximab / Q000627:therapeutic use*; D013997:Time Factors
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D005938:Glucocorticoids; D007155:Immunologic Factors; D000069283:Rituximab; D011239:Prednisolone
keywords: B cells; Systemic vasculitis; Treatment