title: Clinicopathological features of IgG4-related disease complicated with orbital involvement.
abstract: OBJECTIVE
  IgG4-related disease (IgG4-RD) is characterized by IgG4-positive plasmacytic infiltration and fibrosis in various organs. Orbital involvement in IgG4-RD includes lacrimal glands, extra-ocular muscles, trigeminal nerve and other parts of the orbit. Immunohistochemical staining is used to diagnose IgG4-RD in patients with orbital inflammation. The purpose of this retrospective study was to clarify the clinicopathological features of IgG4-RD complicated with orbital involvement.
  
  
  METHODS
  We examined the clinical features, pathological findings and response to treatment in nine patients with IgG4-RD who underwent orbital tissue biopsy between April 2010 and August 2012 at the University of Tsukuba Hospital.
  
  
  RESULTS
  Among the nine patients, eight had dacryoadenitis, one had infraorbital nerve swelling, and another one had IgG4-related orbital inflammation. Involvement of other organs was identified in all patients, including involvement of the salivary glands, lymph nodes, lung, kidney and para-aorta. In all patients, biopsy samples from orbital tissues showed lymphoplasmacytic infiltration and fibrosis, and IgG4-positive/IgG-positive plasmacyte ratio of > 40%. All patients were treated with prednisolone (0.6 mg/kg/day) and responded well in early phase, although relapse was noted in two patients following tapering of prednisolone, evident by swelling of lacrimal glands.
  
  
  CONCLUSIONS
  Patients with IgG4-RD complicated with orbital involvement often present with involvement of other organs. The histopathological findings of orbital tissue match the characteristic features of IgG4-RD. Corticosteroid is effective for orbital and systemic involvement in IgG4-RD.
authors.affiliation: Departments of Internal Medicine, Faculty of Medicine, University of Tsukuba , Tsukuba , Japan.
authors.forename: Chihiro
authors.initials: C
authors.lastname: Hagiya
authors.forename: Hiroto
authors.initials: H
authors.lastname: Tsuboi
authors.forename: Masahiro
authors.initials: M
authors.lastname: Yokosawa
authors.forename: Shinya
authors.initials: S
authors.lastname: Hagiwara
authors.forename: Tomoya
authors.initials: T
authors.lastname: Hirota
authors.forename: Chinatsu
authors.initials: C
authors.lastname: Takai
authors.forename: Hiromitsu
authors.initials: H
authors.lastname: Asashima
authors.forename: Haruka
authors.initials: H
authors.lastname: Miki
authors.forename: Naoto
authors.initials: N
authors.lastname: Umeda
authors.forename: Masanobu
authors.initials: M
authors.lastname: Horikoshi
authors.forename: Yuya
authors.initials: Y
authors.lastname: Kondo
authors.forename: Makoto
authors.initials: M
authors.lastname: Sugihara
authors.forename: Hiroshi
authors.initials: H
authors.lastname: Ogishima
authors.forename: Takeshi
authors.initials: T
authors.lastname: Suzuki
authors.forename: Takahiro
authors.initials: T
authors.lastname: Hiraoka
authors.forename: Yuichi
authors.initials: Y
authors.lastname: Kaji
authors.forename: Isao
authors.initials: I
authors.lastname: Matsumoto
authors.forename: Tetsuro
authors.initials: T
authors.lastname: Oshika
authors.forename: Takayuki
authors.initials: T
authors.lastname: Sumida
mesh_terms: D000368:Aged; D001327:Autoimmune Diseases / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D005128:Eye Diseases / Q000097:blood / Q000175:diagnosis* / Q000473:pathology; D005260:Female; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood*; D008297:Male; D008875:Middle Aged; D009915:Orbit / Q000473:pathology*; D012189:Retrospective Studies
chemical_list: D007074:Immunoglobulin G