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Immunoglobulin G4 Related Lung Disease
Ekrem Cengiz Seyhan1, Mehmet Akif Özgül1, Efsun Uğur1
1Department of Chest Diseases, Ministry of Health, Yedikule Chest Diseases and Thoracic Surgery Training and Research Hospital, İstanbul, Turkey.
Turkish Thoracic Journal
|July 21, 2020
Summary
Immunoglobulin G4 related disease (IgG4-RD) is a multi-organ condition. This case report details a successful glucocorticoid treatment for a patient with IgG4-RD involving the lungs, mediastinum, aorta, and pancreas.
Area of Science:
- Immunology
- Pathology
- Radiology
Background:
- Immunoglobulin G4 related disease (IgG4-RD) is a newly recognized fibroinflammatory condition affecting multiple organs.
- Histopathological hallmarks include lymphoplasmacytic infiltration, fibrosis, and increased IgG4-positive plasma cells.
- Pulmonary manifestations of IgG4-RD can involve the lung parenchyma, airways, pleura, and mediastinum.
Purpose of the Study:
- To report a case of IgG4-RD with extensive multi-organ involvement.
- To highlight the diagnostic criteria and successful treatment of this rare condition.
Main Methods:
- Case report of a patient diagnosed with IgG4-RD.
- Diagnostic evaluation included histopathological examination and serum IgG4 level assessment.
- Treatment involved glucocorticoids.
Main Results:
- The patient presented with involvement of the lung parenchyma, mediastinum, aorta wall, and pancreas.
- Histopathological findings and elevated serum IgG4 levels confirmed the diagnosis of IgG4-RD.
- The patient achieved successful treatment outcomes with glucocorticoids.
Conclusions:
- IgG4-RD is a systemic disease with diverse organ manifestations.
- Accurate histopathological assessment and elevated serum IgG4 levels are crucial for diagnosis.
- Glucocorticoids are an effective first-line treatment for IgG4-RD.
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