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Granulomatosis With Polyangiitis in Otolaryngologist Practice: A Review of Current Knowledge

Joanna Wojciechowska|Wojciech Krajewski|Piotr Krajewski|Tomasz Kręcicki
대한이비인후과학회 (Korean Society of Otorhinolaryngology-Head and Neck Surgery)|2016.03|Clinical and Experimental Otorhinolaryngology|제9권 제1호|pp. 8-13

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참고문헌

키워드

OtolaryngologyGranulomatosis with PolyangiitisWegener Granulomatosis

초록

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.

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.

참고문헌 (51)

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관련 논문AI추천

A Case of Unilateral Otologic Symptoms as Initial Manifestations of Granulomatosis With PolyangiitisBatinović Franko, Durdov Merica Glavina, Martinić Marina Krnić, Sunara Davor대한청각학회 · Journal of Audiology & Otology2023 · 제27권 제3호Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness김상훈, 김수일, 여승근, 정아라대한청각학회 · Journal of Audiology & Otology2016 · 제20권 제1호중이염으로 내원한 Wegener씨 육아종 1예구평모, 김재룡, 이여명, 허경욱대한이비인후과학회 부산,울산,경남 지부회 · 임상이비인후과2016 · 제27권 제1호중이염을 주소로 내원한 ANCA 음성 제한성 육아종증 다발 혈관염 1례문지원, 송재준, 이진환, 채성원대한이비인후과학회 부산,울산,경남 지부회 · 임상이비인후과2021 · 제32권 제1호Wegener’s Granulomatosis Presenting as Bilateral Otalgia with Facial Palsy: A Case Report명나혜, 정재윤대한청각학회 · Journal of Audiology & Otology2013 · 제17권 제1호Wegener 육아종증의 진단과 치료에 대한 임상적 경험공일규, 김대우, 김동영, 김시환, 민양기, 박경태, 이재서, 이철희, 한두희대한이비인후과학회 · 대한이비인후과학회지 두경부외과학2008 · 제51권 제12호

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