title: Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge.
abstract: Granulomatosis with polyangiitis (GPA) is an idiopathic vasculitis of medium and small arteries, characterized by necrotizing granulomatous inflammation. GPA typically affects upper and lower respiratory tract with coexisting glomerulonephritis. This disease is generally characterized by antineutrophil cytoplasm antibodies (ANCA), nevertheless, there are rare cases with negative ANCA. GPA affects people at any age, with predominance of the sixth and seventh decade of life. In 80%-95% of the patients the first symptoms of GPA are otorhinolaryngological manifestations of head and neck including nose/sinuses, ears, eyes, larynx/trachea, oral cavity, and salivary glands. Diagnosis of GPA is based on Criteria of the American College of Rheumatology. In clinical practice diagnosis, the presence of distinctive ANCA antibodies and biopsy of affected organ are crucial. GPA must be differentiated from neoplastic, infectious or inflammatory ulcerative lesions of the head and neck. The standard treatment procedure is divided into two essential phases, induction and maintenance. The induction phase is based on combination of systemic corticosteroid and immunosuppressant therapy, whereas the maintenance phase comprises corticosteroids and azathioprine/methotrexate supplementation. Surgical treatment ought to be considered for patients who are not responding to pharmacotherapy.
authors.affiliation: Department and Clinic of Otolaryngology-Head and Neck Surgery, Wrocław, Poland.
authors.forename: Joanna
authors.initials: J
authors.lastname: Wojciechowska
authors.affiliation: Department of Urology, Wrocław Medical University, Wrocław, Poland.
authors.forename: Wojciech
authors.initials: W
authors.lastname: Krajewski
authors.affiliation: Department of Urology, Wrocław Medical University, Wrocław, Poland.
authors.forename: Piotr
authors.initials: P
authors.lastname: Krajewski
authors.affiliation: Department and Clinic of Otolaryngology-Head and Neck Surgery, Wrocław, Poland.
authors.forename: Tomasz
authors.initials: T
authors.lastname: Kręcicki
keywords: Granulomatosis with Polyangiitis; Otolaryngology; Wegener Granulomatosis