Citation, DOI, disclosures and article data
Citation:
Weerakkody Y, Ibrahim D, Al Salam H, et al. IgG4-related lung disease. Reference article, Radiopaedia.org (Accessed on 14 Mar 2025) https://doi.org/10.53347/rID-29369
IgG4-related lung disease is a recently described condition. It may occur with or without systemic involvement. It is considered part of the spectrum of IgG4-related disease.
Radiographic features
CT
On HRCT of the chest, it may be categorized into four major subtypes 5:
solid nodular subtype
round-shaped ground-glass opacities: showing multiple rounded ground-glass opacities
alveolar interstitial subtype: showing honeycombing, bronchiectasis and diffuse ground glass opacity
bronchovascular subtype: showing thickening of bronchovascular bundles and interlobular septa
Other intrathoracic intrapulmonary features include
Complications
Several reports suggest that those with IgG4-RD may having higher propensity for pulmonary and extrapulmonary malignancies, inclusive of lymphoma, for unknown reasons 6.
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1. Shigemitsu H & Koss M. IgG4-Related Interstitial Lung Disease: A New and Evolving Concept. Curr Opin Pulm Med. 2009;15(5):513-6. doi:10.1097/MCP.0b013e32832ea509 - Pubmed
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2. Yamashita K, Haga H, Kobashi Y, Miyagawa-Hayashino A, Yoshizawa A, Manabe T. Lung Involvement in IgG4-Related Lymphoplasmacytic Vasculitis and Interstitial Fibrosis: Report of 3 Cases and Review of the Literature. Am J Surg Pathol. 2008;32(11):1620-6. doi:10.1097/PAS.0b013e318172622f - Pubmed
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3. Takato H, Yasui M, Ichikawa Y et al. Nonspecific Interstitial Pneumonia with Abundant IgG4-Positive Cells Infiltration, Which Was Thought as Pulmonary Involvement of IgG4-Related Autoimmune Disease. Intern Med. 2008;47(4):291-4. doi:10.2169/internalmedicine.47.0411 - Pubmed
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4. Raj R. IgG4-Related Lung Disease. Am J Respir Crit Care Med. 2013;188(5):527-9. doi:10.1164/rccm.201306-1121ED - Pubmed
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5. Inoue D, Zen Y, Abo H et al. Immunoglobulin G4-Related Lung Disease: CT Findings with Pathologic Correlations. Radiology. 2009;251(1):260-70. doi:10.1148/radiol.2511080965 - Pubmed
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6. Campbell S, Rubio E, Loschner A. Clinical Review of Pulmonary Manifestations of IgG4-Related Disease. Ann Am Thorac Soc. 2014;11(9):1466-75. doi:10.1513/AnnalsATS.201403-128FR - Pubmed
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