title: Granulomatosis with polyangiitis presenting as an orbital inflammatory pseudotumor: a case report.
abstract: BACKGROUND
  Granulomatosis with polyangiitis is a systemic inflammatory disease that often presents with necrosis, granuloma formation and vasculitis of small- to medium-sized vessels. Affected patients usually present with disease of the upper respiratory tract, lungs and kidneys, but this disease has been reported to involve almost any organ. We report the case of a patient with ocular manifestations of granulomatosis with polyangiitis after the remission of renal and auditory manifestations.
  
  
  METHODS
  An 81-year-old Japanese woman had a four-year history of biopsy-proven antineutrophil cytoplasmic antibody-related glomerulonephritis that had been treated with oral prednisolone and was in serological remission. She had also recovered from a one-year history of complete hearing loss immediately following the steroid treatment for glomerulonephritis. She gradually experienced right eye visual disturbance and exophthalmos over a two-month period. Radiographic and histopathological findings revealed an orbital inflammatory pseudotumor. The administration of prednisolone completely restored her right eye visual acuity and eye movement after two weeks. Considering this case retrospectively, our patient had an orbital inflammatory pseudotumor caused by granulomatosis with polyangiitis including a medical history of reversible hearing loss, although her glomerulonephritis had remitted with an undetectable level of specific antineutrophil cytoplasmic antibody.
  
  
  CONCLUSIONS
  In this patient, hearing loss and visual loss occurred at different times during the course of treatment of granulomatosis with polyangiitis. Clinicians should consider a differential diagnosis of granulomatosis with polyangiitis in patients with treatable hearing and visual loss.
authors.affiliation: Department of Internal Medicine, Ako City Hospital, 1090 Nakahiro, Ako, Hyogo, 6780-232, Japan. issenaohi@yahoo.co.jp.
authors.forename: Naohi
authors.initials: N
authors.lastname: Isse
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Nagamatsu
authors.forename: Naoko
authors.initials: N
authors.lastname: Yoshimatsu
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Obata
authors.forename: Noriko
authors.initials: N
authors.lastname: Takahara