Korean J Intern Med.  2019 Jan;34(1):220-226. 10.3904/kjim.2016.304.

Clinical outcomes and pathological characteristics of immunoglobulin G4-related ophthalmic disease versus orbital inflammatory pseudotumor

Affiliations
  • 1Division of Rheumatology, Department of Internal Medicine, College of Medicine, The Catholic University of Korea, Seoul, Korea. rapark@catholic.ac.kr
  • 2Department of Hospital Pathology, College of Medicine, The Catholic University of Korea, Seoul, Korea.
  • 3Department of Ophthalmology, College of Medicine, The Catholic University of Korea, Seoul, Korea.

Abstract

BACKGROUND/AIMS
This study investigated the clinical and pathological features of immunoglobulin G4 (IgG4)-related ophthalmic disease. To clarify the features, we compared IgG4-related ophthalmic disease and orbital inflammatory pseudotumor.
METHODS
We retrospectively reviewed the medical records of 103 patients who were initially diagnosed with orbital inflammatory pseudotumor, and identified 16 cases in which the diagnosis was based on surgical biopsy and for which data in medical records were sufficient for analysis. Immunohistochemical staining of pathological specimens for IgG and IgG4 was performed. Finally, six of IgG4-related ophthalmic disease patient and 10 of orbital inf lammatory pseudotumor patient were analyzed.
RESULTS
The IgG4-related ophthalmic disease group had more IgG4-positive plasma cells and a higher IgG4/IgG plasma cell ratio than the orbital inflammatory pseudotumor group. Collagenous fibrosis and lacrimal gland involvement were significantly more frequent in the IgG4-related ophthalmic disease group. Dense lymphocyte infiltration, obliterative phlebitis, and bilateral lesions were more frequent in IgG4-related ophthalmic disease, but the differences were not significant. The recurrence-free period was shorter in the IgG4-related ophthalmic disease group (p = 0.035).
CONCLUSIONS
The location of the lesion (lacrimal gland), count and ratio of IgG4-positive plasma cells, and collagenous fibrosis aid the diagnosis of IgG4-related ophthalmic disease in patients with idiopathic orbital mass-like lesions. In addition, maintenance therapy should be considered in patients with IgG4-related ophthalmic disease to prevent recurrence.

Keyword

IgG4-related ophthalmic disease; Collagenous fibrosis; Recurrence; Clinical outcome

MeSH Terms

Biopsy
Collagen
Diagnosis
Fibrosis
Humans
Immunoglobulin G
Immunoglobulins*
Lacrimal Apparatus
Lymphocytes
Medical Records
Orbit*
Orbital Pseudotumor*
Phlebitis
Plasma Cells
Recurrence
Retrospective Studies
Collagen
Immunoglobulin G
Immunoglobulins
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