Thoracic manifestations of IgG4 ‐related disease
Article Swipe
R. Müller
,
Mikaël Ebbo
,
Paul Habert
,
Laurent Daniel
,
Antoine Briantais
,
Pascal Chanez
,
Jean Gaubert
,
N. Schleinitz
·
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.1111/resp.14422
YOU?
·
· 2022
· Open Access
·
· DOI: https://doi.org/10.1111/resp.14422
Immunoglobulin G4‐related disease (IgG4‐RD) is a recently described rare systemic fibroinflammatory disease with an estimated incidence of less than 1 in 100,000 persons per year. The disease can affect virtually any organ and is characterized by unifying histopathological findings. Recently, four subgroups of patients have been characterized: hepatobiliary, head and neck, Mikulicz syndrome and retroperitoneal fibrosis, who illustrate the mainly abdominal and ENT tropism of the disease. Yet, thoracic involvement is not uncommon. It can be detected in up to 30% of patients with systemic IgG4‐RD and is the exclusive manifestation of the disease in about 10% of cases. Clinical symptoms are nonspecific and may include dyspnoea, cough or chest pain. Chest CT findings are heterogeneous and primarily include peribronchovascular thickening, nodules, ground‐glass opacities and lymphadenopathy. There is no specific diagnostic test for IgG4‐RD thoracic involvement, which may mimic malignancy or vasculitis. Therefore, a cautious approach is needed to make an accurate diagnosis: a search for extra‐thoracic manifestations, elevated serum IgG4 levels, circulating levels of plasmablasts and pathologic evidence of disease is warranted. Although very suggestive, neither the presence of a polyclonal IgG4 lymphoplasmacytic infiltrate, storiform fibrosis or obliterative phlebitis are sufficient to confirm the histological diagnosis. Steroids are recommended as first‐line therapy. Rituximab or disease‐modifying antirheumatic drugs may be used in relapsed or rare cases of steroid‐refractory disease. In this review, we summarize current knowledge regarding the pathophysiology, epidemiology, diagnostic modalities (clinical–biological–imaging–histopathology) and treatment of IgG4‐RD thoracic involvement.
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- Type
- review
- Language
- en
- Landing Page
- https://doi.org/10.1111/resp.14422
- https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/resp.14422
- OA Status
- hybrid
- Cited By
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- References
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- Related Works
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- OpenAlex ID
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All OpenAlex metadata
Raw OpenAlex JSON
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https://openalex.org/W4310261800Canonical identifier for this work in OpenAlex
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https://doi.org/10.1111/resp.14422Digital Object Identifier
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Thoracic manifestations of
IgG4 ‐related diseaseWork title - Type
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reviewOpenAlex work type
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enPrimary language
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2022Year of publication
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2022-11-27Full publication date if available
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R. Müller, Mikaël Ebbo, Paul Habert, Laurent Daniel, Antoine Briantais, Pascal Chanez, Jean Gaubert, N. SchleinitzList of authors in order
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https://doi.org/10.1111/resp.14422Publisher landing page
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https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/resp.14422Direct link to full text PDF
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hybridOpen access status per OpenAlex
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https://onlinelibrary.wiley.com/doi/pdfdirect/10.1111/resp.14422Direct OA link when available
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Medicine, IgG4-related disease, Rituximab, Disease, Malignancy, Retroperitoneal fibrosis, Pathology, Fibrosis, Dermatology, LymphomaTop concepts (fields/topics) attached by OpenAlex
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32Total citation count in OpenAlex
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2025: 16, 2024: 9, 2023: 5, 2022: 1Per-year citation counts (last 5 years)
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10Other works algorithmically related by OpenAlex
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