title: Microscopic polyangiitis: Advances in diagnostic and therapeutic approaches.
abstract: Microscopic polyangiitis (MPA) is an idiopathic autoimmune disease characterized by systemic vasculitis. The disease predominantly affects small-calibre blood vessels and is associated with the presence of antineutrophil cytoplasmic autoantibodies (ANCA). Microscopic polyangiitis was considered to be a disease entity by Savage et al. in 1985. Microscopic polyangiitis has a reported low incidence and a slight male predominance. The aetiology of MPA remains unknown. There is, however, increased evidence that MPA is an autoimmune disease in which ANCAs, particularly those reacting with MPO, are pathogenic. MPA belongs to the systemic vasculitides, indicating that multiple organs can be affected. The major organs involved in MPA are the kidneys and the lungs. As expected for an illness that affects multiple organ systems, patients with MPA can present with a myriad of different symptoms. Ear, nose and throat (ENT) manifestations are not considered to be clinical symptoms of MPA, but in the majority of populations described, ENT involvement was found in surprisingly high percentages. MPA is part of the ANCA-associated vasculitides, which are characterized by necrotizing vasculitis of small vessels. Diagnosis is mainly established by clinical manifestations, computed tomography (TC), ANCA antibody detection and renal and pulmonary biopsy. The introduction of aggressive immunosuppressive treatment has substantially improved the prognosis. The standardized therapeutic regimen is based on cyclophosphamide and corticosteroids. Using this regimen, remission can be achieved in most of the patients. Rituximab may represent an important alternative to cyclophosphamide for patients who may not respond adequately to antimetabolite therapies.
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 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 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.
authors.forename: Maria Ida
authors.initials: MI
authors.lastname: Rizzo
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: Giuseppe
authors.initials: G
authors.lastname: Magliulo
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, ENT Section, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy.
authors.forename: Mario
authors.initials: M
authors.lastname: Tombolini
authors.affiliation: Department Organs of Sense, ENT Section, University of Rome "La Sapienza", Viale del Policlinico 155, 00100 Roma, Italy.
authors.forename: Rosaria
authors.initials: R
authors.lastname: Turchetta
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: D000818:Animals; D001323:Autoantibodies / Q000627:therapeutic use; D003937:Diagnosis, Differential; D006801:Humans; D055953:Microscopic Polyangiitis / Q000175:diagnosis / Q000453:epidemiology / Q000628:therapy*; D011379:Prognosis; D012074:Remission Induction
chemical_list: D001323:Autoantibodies
keywords: Antineutrophil cytoplasmic autoantibodies; Autoimmune vasculitis; Microscopic polyangiitis; Myeloperoxidase; Proteinase 3