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Refractory Granulomatosis with Polyangiitis Presenting as Facial Paralysis and Bilateral Sudden Deafness

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초록

Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.

Abstract

Granulomatosis with polyangiitisis [(GPA) or Wegener granulomatosis] is a multi-system disease characterized by granuloma formation and necrotizing vasculitis. GPA classically shows involvement of the respiratory tracts and the renal system. However, locoregional disease is common and may include otologic manifestations. Although otologic involvement can occur during the course of GPA, no report has described facial palsy with sudden sensorineural total deafness with vertigo as the presenting feature of GPA. This case describes a patient with multiorgan involving resistant form of GPA initially presenting with bilateral profound sudden sensorineural hearing loss and left facial paralysis with vertigo. The condition responded well to treatment with rituximab.

참고문헌 (9)

  1. Oristrell J, "Effectiveness of rituximab in severe Wegener’s granulomatosis:report of two cases and review of the literature", Open Respir Med J, 3, pp.94~99 (2009)
  2. Erickson VR, "Wegener’s granulomatosis: current trends in diagnosis and management", Curr Opin Otolaryngol Head Neck Surg, 15, pp.170~176 (2007)
  3. Takagi D, "Otologic manifestations of Wegener’s granulomatosis", Laryngoscope, 112, pp.1684~1690 (2002)
  4. Sánchez-Cano D, "Effect of rituximab on refractory Wegener granulomatosis with predominant granulomatous disease", J Clin Rheumatol, 14, pp.92~93 (2008)
  5. Wierzbicka M, "The otologic manifestation of Wegener’s granulomatosis--review of contemporary achievements in diagnostics and treatment", Otolaryngol Pol, 66, pp.254~258 (2012)
  6. Bakthavachalam S, "Hearing loss in Wegener’s granulomatosis", Otol Neurotol, 25, pp.833~837 (2004)
  7. Ferri E, "Early diagnosis of Wegener’s granulomatosis presenting with bilateral facial paralysis and bilateral serous otitis media", Auris Nasus Larynx, 34, pp.379~382 (2007)
  8. Hern JD, "Early diagnosis of Wegener’s granulomatosis presenting with facial nerve palsy", J Laryngol Otol, 110, pp.459~461 (1996)
  9. Keogh KA, "Rituximab for refractory Wegener’s granulomatosis:report of a prospective, open-label pilot trial", Am J Respir Crit Care Med, 173, pp.180~187 (2006)

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