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Updated: Oct 17, 2025
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Published on: June 16, 2020
Thoracic involvement and imaging patterns in IgG4-related disease
Romain Muller1,2, Paul Habert3,2, Mikael Ebbo1
1Dept of Internal Medicine, Aix Marseille University, APHM, CHU La Timone, Marseille, France.
Immunoglobulin G4-related disease (IgG4-RD) thoracic involvement is diverse, often involving peribronchovascular thickening and lymph nodes. High-resolution CT scans reveal varied patterns in Caucasian patients, suggesting this manifestation is underestimated.
Area of Science:
- Radiology
- Immunology
- Rheumatology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic fibroinflammatory condition.
- Thoracic involvement in IgG4-RD is documented but primarily in Asian populations.
- High-resolution thoracic computed tomography (CT) is crucial for assessing IgG4-RD manifestations.
Purpose of the Study:
- To characterize thoracic involvement in Caucasian patients with IgG4-RD using high-resolution CT.
- To identify common CT patterns and their associations in this patient cohort.
Main Methods:
- Retrospective analysis of thoracic CT scans from 48 Caucasian IgG4-RD patients.
- Scans were reviewed by two blinded thoracic imaging experts.
- Patients met stringent diagnostic criteria for IgG4-RD.
Main Results:
- Heterogeneous CT findings were observed, with peribronchovascular involvement (56%) and lymphadenopathy (31%) being most frequent.
- Other patterns included nodular disease, interstitial disease, ground-glass opacities, pleural disease, and fibrosis.
- Asthma showed a significant association with peribronchovascular involvement (p=0.04).
Conclusions:
- Thoracic involvement in IgG4-RD is heterogeneous and potentially underdiagnosed.
- Peribronchovascular thickening and thoracic lymph node enlargement are the predominant CT findings.
- Further research is needed to understand the full spectrum and implications of IgG4-RD thoracic disease.
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