IgG4-related mediastinitis

Orpha code: 63999OMIM code:

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.

Disease data
Classification

Clinical subtype

Synonyms
Fibrosing mediastinitis
Stwardnienie śródpiersia
Mediastinal fibrosis
Sclerosing mediastinitis
ORPHA code
63999
OMIM code
-
ICD10 code
J98.5
ICD11 code
CB22.0

No additional description.

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