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IgG4-related mediastinitis
Disease definition
A rare systemic autoimmune disease characterized by an aggressive fibroinflammatory process with infiltration of IgG4-positive plasma cells in the mediastinum, potentially resulting in compression and functional impairment of vital mediastinal structures, and associated with elevated serum IgG4. Clinical symptoms are unspecific and include pain or symptoms due to mass effect. The condition may occur together with IgG4-related disease in other parts of the body.
ORPHA:63999
Classification level: Subtype of disorderA summary on this disease is available in Deutsch (2020) Español (2020) Français (2020) Nederlands (2020)
Detailed information
General public
- Article for general public
- Français (2014) - SNFMI
- Svenska (2016) - Socialstyrelsen
Disease review articles
- Review article
- English (2014) - Orphanet J Rare Dis
- Diagnostic criteria
- English (2014) - Orphanet J Rare Dis


Additional information
Further information on this disease
Patient-centred resources for this disease
Research activities on this disease
Specialised Social Services
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