중이염을 주소로 내원한 ANCA 음성 제한성 육아종증 다발 혈관염 1례 = A Case of ANCA-Negative Limited Granulomatosis with Polyangiitis Presenting with Otitis Media

다국어 초록 (Multilingual Abstract)

Granulomatosis with polyangiitis(GPA) is a systemic disease that causes granulomatous inflammation and vasculitis to small to medium sized vessels. Although prompt diagnosis is vital for the successful treatment of the disease, it is often misdiagnosed as a simple inflammatory disease in ENT clinic. Anti-neutrophil cytoplasmic antibody(ANCA) tests positive in up to 90% of GPA, and it is a useful diagnostic marker for its strong association with the disease. However, we experienced ANCA-negative limited GPA presenting with intractable otitis media, thereby reporting this case with a brief review of the literature.

참고문헌 (Reference)

1 Gottschlich S, "Wegener’s granulomatosis in the head and neck region" 52 (52): 935-945, 2004

2 Ananthakrishnan L, "Wegener’s granulomatosis in the chest : high-resolution CT findings" 192 (192): 676-682, 2009

3 De Groot K, "Wegener’s granulomatosis : disease course, assessment of activity and extent and treatment" 7 (7): 285-291, 1998

4 Verma N, "Wegener’s granulomatosis : an unusual presentation case report and review of the literature" 14 (14): 2012

5 Lamprecht P, "Wegener’s granulomatosis" 29 (29): 47-56, 2004

6 박경태, "Wegener 육아종증의 진단과 치료에 대한 임상적 경험" 대한이비인후과학회 51 (51): 1109-1118, 2008

7 Bosch X, "Treatment of antineutrophil cytoplasmic antibody–associated vasculitis : a systematic review" 298 (298): 655-669, 2007

8 Leavitt RY, "The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis" 33 (33): 1101-1107, 1990

9 Schönermarck U, "Prevalence and spectrum of rheumatic diseases associated with proteinase 3-antineutrophil cytoplasmic antibodies(ANCA)and myeloperoxidase-ANCA" 40 (40): 178-184, 2001

10 Joglekar S, "Polyarteritis nodosa : a human temporal bone study" 31 (31): 221-225, 2010

11 Nishino H, "Neurological involvement in Wegener’s granulomatosis : an analysis of 324 consecutive patients at the Mayo Clinic" 33 (33): 4-9, 1993

12 Maranhão ASdA, "Mastoiditis and facial paralysis as initial manifestations of Wegener’s granulomatosis" 78 (78): 80-86, 2012

13 Devaney KO, "Interpretation of head and neck biopsies in Wegener’s granulomatosis. A pathologic study of 126 biopsies in 70 patients" 14 (14): 555-564, 1990

14 Bakthavachalam S, "Hearing loss in Wegener’s granulomatosis" 25 (25): 833-837, 2004

15 Lidar M, "Hearing loss as the presenting feature of systemic vasculitis" 1107 (1107): 136-141, 2007

16 Roszkowska A, "Bilateral facial palsy in rapidly progressive course of Wegener’s granulomatosis: a case report" 2013

17 Ozaki S, "ANCA-associated vasculitis : diagnostic therapeutic strategy" 56 (56): 87-96, 2007

18 Finkielman JD, "ANCA are detectable in nearly all patients with active severe Wegener’s granulomatosis" 120 (120): 643. e9-14, 2007