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Updated: May 11, 2026

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Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
Radiographic patterns of orbital involvement in IgG4-related disease
Daniel T Ginat1, Suzanne K Freitag, David Kieff
1Department of Radiology, Massachusetts General Hospital, Boston, Massachusetts 02114, USA. ginatd01@gmail.com
Ophthalmic Plastic and Reconstructive Surgery
|May 7, 2013
Summary
Immunoglobulin G4 (IgG4)-related disease shows diverse orbital imaging patterns, including lacrimal gland and muscle involvement. Extraorbital disease is common and aids in diagnosis.
Area of Science:
- Ophthalmology
- Radiology
- Immunology
Background:
- Immunoglobulin G4 (IgG4)-related disease is a systemic condition.
- It can manifest with lymphoplasmacytic infiltrates in the orbit.
Purpose of the Study:
- To identify and characterize the imaging patterns of orbital IgG4-related disease.
Main Methods:
- Retrospective review of CT, MRI, and PET scans in patients with confirmed IgG4-related disease.
- Analysis of imaging findings in 9 patients with orbital involvement.
Main Results:
- Orbital IgG4-related disease presented with varied patterns: lacrimal gland/sac involvement, extraocular muscle thickening, preseptal/orbital fat/cranial nerve involvement.
- CT revealed orbital wall demineralization in 2 cases.
- MRI showed diverse signal intensities and diffuse enhancement.
- PET indicated hypermetabolic lesions in most cases.
- Extraorbital involvement was present in 6 of 9 patients.
Conclusions:
- Orbital IgG4-related disease exhibits a broad spectrum of imaging manifestations.
- The presence of extraorbital disease is frequent and can be a key diagnostic clue.
