안면 마비와 난청으로 진단된 면역글로불린 G4 연관질환 동반 육아종성 다발 혈관염 1예
A Case of Granulomatosis with Polyangiitis with IgG4-Related Disease Expressed by Facial Paralysis and Hearing Loss
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초록
Granulomatosis with polyangiitis (GPA) and Immunoglobulin (Ig) G4-related disease (IgG4-RD) are rare diseases and early diagnosis and proper management are imperative to prevent multi-organ damage. The authors present a case of a 60 years old woman who had facial paralysis and hearing loss. Lt intact canal wall tympanomastoidectomy, Lt facial nerve decompression and ossiculoplasty with partial ossicular replacement prosthesis (PORP) was done. During operation, middle ear tissue was biopsied and GPA with IgG4-RD was diagnosed. After methyl prednisolone (MPD) pulse therapy and azathioprine therapy, the severity of paralysis was improved. We present this case because common otologic symptoms like facial palsy and hearing loss could be initial symptoms of rare systemic disease
Abstract
Granulomatosis with polyangiitis (GPA) and Immunoglobulin (Ig) G4-related disease (IgG4-RD) are rare diseases and early diagnosis and proper management are imperative to prevent multi-organ damage. The authors present a case of a 60 years old woman who had facial paralysis and hearing loss. Lt intact canal wall tympanomastoidectomy, Lt facial nerve decompression and ossiculoplasty with partial ossicular replacement prosthesis (PORP) was done. During operation, middle ear tissue was biopsied and GPA with IgG4-RD was diagnosed. After methyl prednisolone (MPD) pulse therapy and azathioprine therapy, the severity of paralysis was improved. We present this case because common otologic symptoms like facial palsy and hearing loss could be initial symptoms of rare systemic disease
참고문헌 (10)
- Falk RJ, "Granulomatosis with polyangiitis (Wegener’s):an alternative name for Wegener’s granulomatosis", J Am Soc Nephrol, 22(4), pp.587~588 (2011)
- Gottschlich S, "Wegener’s granulomatosis in the head and neck region", HNO, 52(10), pp.935~945 (2004)
- De Groot K, "Standardized neurologic evaluation of 128 patients with Wegener granulomatosis", Arch Neurol, 58, pp.1215~1221 (2001)
- Wallace ZS, "IgG4-Related Disease : Clinical and Laboratory Features in One Hundred Twenty-Five Patients", Arthritis Rheumatol, 67(9), pp.2466~2475 (2015)
- Umehara H, "Comprehensive diagnostic criteria for IgG4-related disease, 2011", Mod Rheumatol, 22, pp.21~30 (2012)
- Umehara H, "A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details", Mod Rheumatol, 22, pp.1~14 (2012)
- Khosroshahi A, "Rituximab for the treatment of IgG4-related disease : lessons from 10 consecutive patients", Medicine, 91, pp.57~ (2012)
- Ebbo M, "Long-term efficacy and safety of rituximab in IgG4-related disease: Data from a French nationwide study of thirty-three patients", PLoS One, 12(9), pp.e0183844~ (2017)
- Yates M, "EULAR", ERA-EDTA recommendations for the management of ANCA-associated vasculitis, 2016, pp.75, 1583~
- Danlos FX, "Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease : a new overlap symdrome", Autoimmun Rev, 16(10), pp.1036~1043 (2017)