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KMID : 0390220180290020240
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Journal of Clinical Otolaryngology, Head and Neck Surgery
2018 Volume.29 No. 2 p.240 ~ p.244
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A Case of Granulomatosis with Polyangiitis with IgG4-Related Disease Expressed by Facial Paralysis and Hearing Loss
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Kim Hyung-Sik
Doo Jeon-Gang
Yeo Seung-Geun
Kim Sang-Hoon
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Abstract
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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.
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KEYWORD
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Granulomatosis with polyangiitis, Immunoglobulin G, Facial nerve palsy
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