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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 disorder
  • Synonym(s):
    • Fibrosing mediastinitis
    • Mediastinal fibrosis
    • Sclerosing mediastinitis
  • Prevalence: -
  • Inheritance: Not applicable 
  • Age of onset: -
  • ICD-10: J98.5
  • ICD-11: CB22.0
  • OMIM: -
  • UMLS: C0264573
  • MeSH: C536136
  • GARD: 8337
  • MedDRA: 10027074

  A summary on this disease is available in Deutsch (2020) Español (2020) Français (2020) Nederlands (2020)

Detailed information

General public

Disease review articles

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

Further information on this disease

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Specialised Social Services

The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.