title: Optimal therapy and prospects for new medicines in eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome).
abstract: BACKGROUND
  The prevalence of eosinophilic granulomatosis with polyangiitis (EGPA; previously known as Churg-Strauss syndrome) is lower than that of other antineutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV's), and only a few randomized controlled trials have been conducted for this rare disease. However, recent international efforts have helped delineate the best treatment approach.
  
  
  METHODS
  At present, EGPA conventional therapy is by default similar to that of other AAVs. Limited, non-severe EGPA can initially be treated with glucocorticoids (GCs) alone. Patients with life-threatening manifestations and/or major organ involvement must receive a combination of GCs and an immunosuppressant, mainly cyclophosphamide. Remission can be achieved in >85% of patients with these first-line treatments, but vasculitis relapses occur in more than one-third of patients, and about 85% cannot stop GC treatment because of GC-dependent asthma and/or ENT manifestations. A few biologic agents, including rituximab or mepolizumab, are now under investigation after interesting preliminary results. Expert commentary: Treatment for EGPA still has several unmet needs. Several biologic agents are now under investigation in randomized controlled trials, but a few others should be considered soon. Their benefit should be demonstrated for devising more EGPA-tailored therapeutic strategies (ideally GC-free).
authors.affiliation: a Division of Rheumatology, Vasculitis Clinic, Mount Sinai Hospital , University Health Network , Toronto , Ontario , Canada.|b CanVasc , Toronto , Ontario , Canada.
authors.forename: Christian
authors.initials: C
authors.lastname: Pagnoux
authors.affiliation: c Department of Internal Medicine, National Referral Center for Rare Autoimmune and Systemic Diseases (Vasculitis, Scleroderma), Hôpital Cochin, APHP , Université Paris Descartes , Paris , France.
authors.forename: Matthieu
authors.initials: M
authors.lastname: Groh
mesh_terms: D000818:Animals; D061067:Antibodies, Monoclonal, Humanized / Q000627:therapeutic use; D015267:Churg-Strauss Syndrome / Q000628:therapy*; D002986:Clinical Trials as Topic; D003520:Cyclophosphamide / Q000627:therapeutic use*; D004804:Eosinophils / Q000187:drug effects* / Q000276:immunology; D005938:Glucocorticoids / Q000627:therapeutic use*; D014890:Granulomatosis with Polyangiitis / Q000628:therapy*; D006801:Humans; D007167:Immunotherapy / Q000379:methods* / Q000639:trends; D012008:Recurrence; D000069283:Rituximab / Q000627:therapeutic use
chemical_list: D061067:Antibodies, Monoclonal, Humanized; D005938:Glucocorticoids; D000069283:Rituximab; D003520:Cyclophosphamide; C434107:mepolizumab
keywords: Churg-Strauss syndrome; Eosinophilic granulomatosis with polyangiitis; cyclophosphamide; glucocorticoids; treatment