IgG4-related mediastinitis
Synonyms: Fibrosing mediastinitis · Mediastinal fibrosis · Sclerosing mediastinitis
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Status
- active
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.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | IgG4-related mediastinitis | Fibrosing mediastinitis · Mediastinal fibrosis · Sclerosing mediastinitis |
| fr | Médiastinite associée à IgG4 | Fibrose médiastinale · Médiastinite fibreuse · Médiastinite sclérosante |
| de | Mediastinitis, IgG4-assoziierte | Mediastinalfibrose · Mediastinitis, fibrosierende · Mediastinitis, sklerosierende |
| es | Mediastinitis asociada a IgG4 | Fibrosis mediastínica · Mediastinitis esclerosante · Mediastinitis fibrosante |
| it | Mediastinite correlata alle IgG4 | Fibrosi mediastinica · Mediastinite fibrosante · Mediastinite sclerosante |
| nl | IgG4-gerelateerde mediastinitis | Fibroserende mediastinitis · Mediastinale fibrose · Scleroserende mediastinitis |
| pt | Mediastinite IgG4-relacionada | Fibrose mediastínica · Mediastinite esclerosante · Mediastinite fibrosante |
| pl | Włóknienie śródpiersia | Stwardnienie śródpiersia |
| cs | IgG4 asociovaná mediastinitida | Fibrotizující mediastinitida · Mediastinální fibróza · Sklerotizující mediastinitida |
| uk | Медіастиніт, повязаний з IgG4 | Фіброзуючий медіастиніт · Фіброз середостіння · Склерозуючий медіастиніт |
| zh | IgG4相关性纵隔炎 | 硬化性纵隔炎 |
| tr | IgG4-ilişkili mediastinit | Mediastinal fibroz · Fibrozan mediastinit · Sklerozan mediastinit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J98.5 | NTBT | inclusion | validated |
| ICD-11 | CB22.0 | E | specific | validated |
| MONDO | 0018978 | E | validated | |
| MeSH | C536136 | E | validated | |
| UMLS | C0264573 | E | validated | |
| GARD | 8337 | E | validated | |
| MedDRA | 10027074 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:596448IgG4-related systemic disease
rare systemic and rheumatological diseases - ORPHA:97955Rare respiratory disease
rare respiratory diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/63999
- This record as JSON: /data/d/63999.json