title: Granulomatosis with polyangiitis and facial palsy: Literature review and insight in the autoimmune pathogenesis.
abstract: Granulomatosis with polyangiitis (GPA) is an autoimmune systemic necrotizing small-vessel vasculitis associated with the presence of anti-neutrophil cytoplasmic antibodies (ANCA). Oto-neurological manifestations of ANCA-associated vasculitis according to PR3-ANCA positivity and MPO-ANCA positivity are usually reported. Facial nerve palsy is usually reported during the clinical course of the disease but it might appear as the presenting sign of GPA. Necrotizing vasculitis of the facial nerve 'vasa nervorum' is nowadays the most widely accepted etiopathogenetic theory to explain facial damage in GPA patients. A central role for PR3-ANCA in the pathophysiology of vasculitis in GPA patients with oto-neurological manifestation is reported. GPA requires prompt, effective management of the acute and chronic manifestations. Once the diagnosis of GPA has been established, clinicians should devise an appropriate treatment strategy for each individual patient, based on current clinical evidence, treatment guidelines and recommendations.
authors: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico,151-00161, Rome, Italy. Electronic address: giannicolaiannella@hotmail.it.
authors: Giannicola
authors: G
authors: Iannella
authors: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico,151-00161, Rome, Italy. Electronic address: antonio.greco@uniroma1.it.
authors: Antonio
authors: A
authors: Greco
authors: Department of Clinical Immunology, Sapienza University of Rome, Viale dell'Università, 37-00161 Rome, Italy. Electronic address: guido.granata@alice.it.
authors: Guido
authors: G
authors: Granata
authors: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico,151-00161, Rome, Italy. Electronic address: alessandramanno@hotmail.it.
authors: Alessandra
authors: A
authors: Manno
authors: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico,151-00161, Rome, Italy. Electronic address: benedetta.pasquariello@gmail.com.
authors: Benedetta
authors: B
authors: Pasquariello
authors: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico,151-00161, Rome, Italy. Electronic address: dilettaangeletti@gmail.com.
authors: Diletta
authors: D
authors: Angeletti
authors: First Dermatology Division, Institute Dermopatico dell'Immacolata-IRCCS, Via Monti di Creta, 104-00167 Rome, Italy. Electronic address: dario.didona@gmail.com.
authors: Dario
authors: D
authors: Didona
authors: Organi di Senso Department University, Sapienza University of Rome, Viale del Policlinico,151-00161, Rome, Italy. Electronic address: giuseppemagliuloorl@yahoo.com.
authors: Giuseppe
authors: G
authors: Magliulo
mesh_terms: D000818:Animals; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000276:immunology; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000378:metabolism*; D020330:Bell Palsy / Q000276:immunology; D018450:Disease Progression; D005158:Facial Paralysis / Q000276:immunology* / Q000473:pathology; D014890:Granulomatosis with Polyangiitis / Q000276:immunology* / Q000473:pathology; D006801:Humans
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic
keywords: ANCA-associated vasculitis; Autoimmune facial palsy; Facial nerve; Facial palsy; Granulomatosis with polyangiitis; Wegener's granulomatosis