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IgG4-related disease in idiopathic sclerosing orbital inflammation
  1. Ho-Seok Sa1,
  2. Ju-Hyang Lee2,
  3. Kyung In Woo2,
  4. Yoon-Duck Kim2
  1. 1Department of Ophthalmology, Asan Medical Center, University of Ulsan, College of Medicine, Seoul, Korea
  2. 2Department of Ophthalmology, Samsung Medical Center, Sungkyunkwan University School of Medicine, Seoul, Korea
  1. Correspondence to Dr Yoon-Duck Kim, Department of Ophthalmology, Samsung Medical Center, 50 Ilwon-dong, Kangnam-ku, Seoul 135-710, Korea; ydkimoph{at}


Aims 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.

  • Lacrimal gland
  • Orbit
  • Pathology
  • Inflammation

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