title: IgG4-related disease in idiopathic sclerosing orbital inflammation.
abstract: OBJECTIVE
  To investigate the frequency of IgG4-related disease (IgG4-RD) among patients previously diagnosed with idiopathic sclerosing orbital inflammation (ISOI), and to compare the clinical features and treatment outcomes of patients with ISOI associated with IgG4-RD and those without IgG4.
  
  
  METHODS
  Retrospective clinicopathological series of 24 patients with ISOI diagnosed between June 2001 and June 2010. Biopsy specimens were immunostained for IgG-expressing and IgG4-expressing cells. Clinical data of patients with IgG4-RD and ISOI unrelated to IgG4 were obtained from patient records.
  
  
  RESULTS
  Of 24 patients, 11 patients (45.8%) were identified with IgG4-RD. 10 patients (10/11, 90.9%) presented with bilateral lacrimal gland enlargement, and seven of those also had submadibular gland enlargement. One patient (1/11, 9.1%) presented with a superior orbital mass. All patients were successfully treated with steroids and/or radiotherapy or had an indolent clinical course. 13 patients (54.2%) were identified with ISOI unrelated to IgG4. Eight patients (8/13, 61.5%) showed unilateral orbital involvement, and nine patients (9/13, 69.2%) had orbital lesions not involving the lacrimal glands. Treatment modalities for ISOI unrelated to IgG4 were varied and less effective: eight patients (61.5%) relapsed following initial treatment with steroids or radiation, and additional therapies were required to enter remission.
  
  
  CONCLUSIONS
  IgG4-RD may be identified frequently in patients with ISOI, and distinguishing features may be bilateral lacrimal gland enlargement with associated submandibular gland enlargement. Patients with IgG4-RD may have better treatment outcomes with less aggressive treatment modalities than those with ISOI unrelated to IgG4. An additional workup for IgG4-RD should be considered in all histopathological biopsy specimens suspicious of ISOI.
authors.affiliation: Department of Ophthalmology, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea.
authors.forename: Ho-Seok
authors.initials: HS
authors.lastname: Sa
authors.affiliation: Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Ju-Hyang
authors.initials: JH
authors.lastname: Lee
authors.affiliation: Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Kyung In
authors.initials: KI
authors.lastname: Woo
authors.affiliation: Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea.
authors.forename: Yoon-Duck
authors.initials: YD
authors.lastname: Kim
mesh_terms: D000328:Adult; D000368:Aged; D001327:Autoimmune Diseases / Q000175:diagnosis / Q000188:drug therapy / Q000209:etiology*; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007074:Immunoglobulin G / Q000276:immunology*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008232:Lymphoproliferative Disorders / Q000175:diagnosis / Q000209:etiology* / Q000628:therapy; D008297:Male; D008875:Middle Aged; D009915:Orbit / Q000473:pathology*; D016727:Orbital Pseudotumor / Q000150:complications* / Q000175:diagnosis / Q000628:therapy; D010950:Plasma Cells / Q000276:immunology; D049268:Positron-Emission Tomography; D011878:Radiotherapy; D012189:Retrospective Studies; D012598:Sclerosis; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G; D007166:Immunosuppressive Agents
keywords: Inflammation; Lacrimal gland; Orbit; Pathology