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IgG4-related disease causing dacryoadenitis, bronchial stenosis and lobar collapse
Jessica Chan1, Kathryn Biddle2, Anna Green3
1Rheumatology, Guy's and St Thomas' NHS Foundation Trust, London, UK jessica.chan@doctors.org.uk.
BMJ Case Reports
|February 13, 2025
Summary
Immunoglobulin G4-related lung disease (IgG4-RLD) is a rare condition. This case highlights IgG4-RLD presenting as bronchial stenosis and lung collapse, expanding understanding of its diverse respiratory manifestations.
Area of Science:
- Rheumatology
- Pulmonology
- Pathology
Background:
- Immunoglobulin G4-related disease (IgG4-RD) is a rare systemic fibroinflammatory condition.
- IgG4-RD can affect virtually any organ, with common sites including salivary glands, pancreas, and retroperitoneum.
- Immunoglobulin G4-related lung disease (IgG4-RLD) is a recognized manifestation, presenting heterogeneously.
Purpose of the Study:
- To describe a unique case of IgG4-RLD.
- To highlight an unusual presentation of IgG4-RLD involving bronchial stenosis and lobar collapse.
Main Methods:
- Case report.
- Clinical case review.
- Diagnostic imaging review.
Main Results:
- A patient presented with bronchial stenosis and left upper lobe collapse.
- The clinical presentation was attributed to Immunoglobulin G4-related lung disease (IgG4-RLD).
- This case expands the spectrum of known IgG4-RLD pulmonary manifestations.
Conclusions:
- IgG4-RLD can present with diverse and severe pulmonary complications, including bronchial stenosis.
- Recognition of these varied presentations is crucial for timely diagnosis and management of IgG4-related lung disease.
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