title: Churg-Strauss syndrome.
abstract: Churg-Strauss syndrome (CSS), alternatively known as eosinophilic granulomatosis with polyangiitis (EGPA), was first described in 1951 by Churg and Strauss as a rare disease characterized by disseminated necrotizing vasculitis with extravascular granulomas occurring exclusively among patients with asthma and tissue eosinophilia. EGPA is classified as a small-vessel vasculitis associated with antineutrophil cytoplasmic antibodies (ANCAs) and the hypereosinophilic syndromes (HESs) in which vessel inflammation and eosinophilic proliferation are thought to contribute to organ damage. Although still considered an idiopathic condition, EGPA is classically considered a Th2-mediated disease. Emerging clinical observations provide compelling evidence that ANCAs are primarily and directly involved in the pathogenesis of AASVs, although recent evidence implicates B cells and the humoral response as further contributors to EGPA pathogenesis. EGPA has traditionally been described as evolving through a prodromic phase characterized by asthma and rhino-sinusitis, an eosinophilic phase marked by peripheral eosinophilia and organ involvement, and a vasculitic phase with clinical manifestations due to small-vessel vasculitis. The American College of Rheumatology defined the classification criteria to distinguish the different types of vasculitides and identified six criteria for EGPA. When four or more of these criteria are met, vasculitis can be classified as EGPA. The French Vasculitis Study Group has identified five prognostic factors that make up the so-called five-factor score (FFS). Patients without poor prognosis factors (FFS=0) have better survival rates than patients with poor prognosis factors (FFS≥1). The treatment of patients with CSS must be tailored to individual patients according to the presence of poor prognostic factors. A combination of high-dose corticosteroids and cyclophosphamide is still the gold standard for the treatment of severe cases, but the use of biological agents such as rituximab or mepolizumab seems to be a promising therapeutic alternative.
authors.affiliation: Department Organs of Sense, ENT Section, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy.
authors.forename: Antonio
authors.initials: A
authors.lastname: Greco
authors.affiliation: Department of Surgical Science, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy.
authors.forename: Maria Ida
authors.initials: MI
authors.lastname: Rizzo
authors.affiliation: Department Organs of Sense, ENT Section, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy; Department of Surgical Science, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy. Electronic address: armando.devirgilio@gmail.com.
authors.forename: Armando
authors.initials: A
authors.lastname: De Virgilio
authors.affiliation: Department of Medico-Surgical Sciences and Biotechnologies, Otorhinolaryngology Section, "Sapienza" University of Rome, Corso della Repubblica, 79, 04100 Latina, LT, Italy.
authors.forename: Andrea
authors.initials: A
authors.lastname: Gallo
authors.affiliation: Department Organs of Sense, ENT Section, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy.
authors.forename: Massimo
authors.initials: M
authors.lastname: Fusconi
authors.affiliation: Department Organs of Sense, ENT Section, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy.
authors.forename: Giovanni
authors.initials: G
authors.lastname: Ruoppolo
authors.affiliation: Department Organs of Sense, Audiology Section, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy.
authors.forename: Giancarlo
authors.initials: G
authors.lastname: Altissimi
authors.affiliation: Department Organs of Sense, ENT Section, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy.
authors.forename: Marco
authors.initials: M
authors.lastname: De Vincentiis
mesh_terms: D000305:Adrenal Cortex Hormones / Q000627:therapeutic use; D000818:Animals; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001249:Asthma / Q000150:complications; D015267:Churg-Strauss Syndrome / Q000188:drug therapy* / Q000453:epidemiology / Q000276:immunology* / Q000503:physiopathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D004804:Eosinophils / Q000473:pathology; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D035583:Rare Diseases / Q000188:drug therapy; D000069283:Rituximab
chemical_list: D000305:Adrenal Cortex Hormones; D019268:Antibodies, Antineutrophil Cytoplasmic; D058846:Antibodies, Monoclonal, Murine-Derived; D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D000069283:Rituximab; D003520:Cyclophosphamide
keywords: ANCA-associated vasculitis; Asthma; Churg–Strauss syndrome; Eosinophilic granulomatosis with polyangiitis; Hypereosinophilic syndromes; Wegener granulomatosis