Journal of Clinical Otolaryngology Head and Neck Surgery (임상이비인후과)

The Busan, Ulsan, Gyeoungnam Branch of Korean Society of Otolaryngology-Head and Neck Surgery (대한이비인후과학회 부산,울산,경남 지부회)
권호 표지

A Case of Granulomatosis with Polyangiitis with IgG4-Related Disease Expressed by Facial Paralysis and Hearing Loss

안면 마비와 난청으로 진단된 면역글로불린 G4 연관질환 동반 육아종성 다발 혈관염 1예

  • Kim, Hyung-sik (Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyung Hee University) ;
  • Doo, Jeon Gang (Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyung Hee University) ;
  • Yeo, Seung Geun (Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyung Hee University) ;
  • Kim, Sang Hoon (Department of Otorhinolaryngology-Head and Neck Surgery, School of Medicine, Kyung Hee University)
  • 김형식 (경희대학교 의과대학.의학전문대학원 이비인후과학교실) ;
  • 두전강 (경희대학교 의과대학.의학전문대학원 이비인후과학교실) ;
  • 여승근 (경희대학교 의과대학.의학전문대학원 이비인후과학교실) ;
  • 김상훈 (경희대학교 의과대학.의학전문대학원 이비인후과학교실)
  • Received : 2018.08.03
  • Accepted : 2018.11.28
  • Published : 2018.12.30

⟨ Previous Next ⟩

Abstract

Granulomatosis with polyangiitis (GPA) and Immunoglobulin (Ig) $G_4$-related disease ($IgG_4$-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 $IgG_4$-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.

Keywords

References

  1. Falk RJ, Gross WL, Guillevin L, Hoffman G, Jayne DR, Jennette JC, el al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. J Am Soc Nephrol. 2011;22(4):587-8. https://doi.org/10.1681/ASN.2011010081
  2. Gottschlich S, Ambrosch P, Gross WL, Hellmich B. Wegener's granulomatosis in the head and neck region. HNO 2004;52(10):935-45. https://doi.org/10.1007/s00106-004-1118-9
  3. De Groot K, Schmidt DK, Arlt AC, Gross WL, Reinhold-Keller E. Standardized neurologic evaluation of 128 patients with Wegener granulomatosis. Arch Neurol 2001;58:1215-21. https://doi.org/10.1001/archneur.58.8.1215
  4. Wallace ZS, Deshpande V, Mattoo H, Mahajan VS, Kulikova M, Pillai S, et al. IgG4-Related Disease: Clinical and Laboratory Features in One Hundred Twenty-Five Patients. Arthritis Rheumatol 2015;67(9):2466-75. https://doi.org/10.1002/art.39205
  5. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. Comprehensive diagnostic criteria for IgG4-related disease, 2011. Mod Rheumatol 2012;22:21-30. https://doi.org/10.3109/s10165-011-0571-z
  6. Umehara H, Okazaki K, Masaki Y, Kawano M, Yamamoto M, Saeki T, et al. A novel clinical entity, IgG4-related disease (IgG4RD): general concept and details. Mod Rheumatol 2012;22:1-14. https://doi.org/10.3109/s10165-011-0508-6
  7. Khosroshahi A, Carruthers MN, Deshpande V, Unizony S, Bloch DB, Stone JH. Rituximab for the treatment of IgG4-related disease: lessons from 10 consecutive patients. Medicine 2012;91:57. https://doi.org/10.1097/MD.0b013e3182431ef6
  8. Ebbo M, Grados A, Samson M, Groh M, Loundou A, Rigolet A, et al. Long-term efficacy and safety of rituximab in IgG4-related disease: Data from a French nationwide study of thirty-three patients. PLoS One 2017;12(9):e0183844. https://doi.org/10.1371/journal.pone.0183844
  9. Yates M, Watts RA, Bajema IM, Cid MC, Crestani B, Hauser T, et al. EULAR/ERA-EDTA recommendations for the management of ANCA-associated vasculitis. Ann Rheum Dis 2016;75:1583.
  10. Danlos FX, Rossi GM, Blockmans D, Emmi G, Kronbichler A, Durupt S et al. Antineutrophil cytoplasmic antibody-associated vasculitides and IgG4-related disease: a new overlap symdrome. Autoimmun Rev 2017;16(10):1036-43. https://doi.org/10.1016/j.autrev.2017.07.020