title: Rituximab for eosinophilic granulomatosis with polyangiitis with severe vasculitic neuropathy: Case report and review of current clinical evidence.
abstract: OBJECTIVE
  Rituximab is approved for the treatment of granulomatosis with polyangiitis and microscopic polyangiitis. Our objective was to review published clinical evidence on the efficacy of rituximab in the treatment of eosinophilic granulomatosis and polyangiitis (EGPA).
  
  
  METHODS
  We describe a case of refractory EGPA with severe vasculitic neuropathy, which responded impressively to B-cell-depleting therapy. A systematic search of the English literature was also performed to capture all available clinical evidence on the use of rituximab in EGPA.
  
  
  RESULTS
  We identified a total of 73 EGPA patients who have been treated with rituximab, all data coming from case series or isolated case reports. The majority of patients (85.1%) were treated for refractory or relapsing disease; a mean (SD) of 2.1 (0.9) different immunosuppressive agents were used prior to rituximab administration. Efficacy of RTX therapy was significant in the majority of cases and in a wide variety of disease manifestations; however, a lack of standardized assessment of disease activity before and after treatment was observed in many reports. Overall, 54.0% of patients were treated with a single cycle of rituximab and only 10.8% experienced relapses of the disease. Few significant side effects were observed during a highly variable period of follow-up (3 months to 5 years), mainly severe infections and allergic reactions.
  
  
  CONCLUSIONS
  RTX seems to be effective in cases of severe EGPA refractory to standard of care immunosuppressive treatment, although support comes from case reports and non-controlled studies.
authors.affiliation: Department of Rheumatology, Clinical Immunology, and Allergy, University Hospital of Heraklion, Stavrakia Voutes, 71110, Heraklion, Crete, Greece. Electronic address: afanouriakis@edu.med.uoc.gr.
authors.forename: Antonis
authors.initials: A
authors.lastname: Fanouriakis
authors.affiliation: Department of Rheumatology, Clinical Immunology, and Allergy, University Hospital of Heraklion, Stavrakia Voutes, 71110, Heraklion, Crete, Greece.
authors.forename: Nikolaos
authors.initials: N
authors.lastname: Kougkas
authors.affiliation: 2nd Department of Medicine, Hippokration General Hospital, Athens University School of Medicine, Athens, Greece.
authors.forename: Dimitrios
authors.initials: D
authors.lastname: Vassilopoulos
authors.affiliation: Department of Rheumatology, Clinical Immunology, and Allergy, University Hospital of Heraklion, Stavrakia Voutes, 71110, Heraklion, Crete, Greece.
authors.forename: Eleni
authors.initials: E
authors.lastname: Fragouli
authors.affiliation: Department of Rheumatology, Clinical Immunology, and Allergy, University Hospital of Heraklion, Stavrakia Voutes, 71110, Heraklion, Crete, Greece.
authors.forename: Argyro
authors.initials: A
authors.lastname: Repa
authors.affiliation: Department of Rheumatology, Clinical Immunology, and Allergy, University Hospital of Heraklion, Stavrakia Voutes, 71110, Heraklion, Crete, Greece.
authors.forename: Prodromos
authors.initials: P
authors.lastname: Sidiropoulos
mesh_terms: D015267:Churg-Strauss Syndrome / Q000188:drug therapy*; D014890:Granulomatosis with Polyangiitis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D000069283:Rituximab / Q000627:therapeutic use*
chemical_list: D007166:Immunosuppressive Agents; D000069283:Rituximab
keywords: Eosinophilic granulomatosis with polyangiitis; Literature review; Rituximab; Vasculitic neuropathy