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.
journal_name
Autoimmun Revjournal_title
Autoimmunity reviewsauthors
Iannella G,Greco A,Granata G,Manno A,Pasquariello B,Angeletti D,Didona D,Magliulo Gdoi
10.1016/j.autrev.2016.02.005subject
Has Abstractpub_date
2016-07-01 00:00:00pages
621-31issue
7eissn
1568-9972issn
1873-0183pii
S1568-9972(16)30031-3journal_volume
15pub_type
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