Eosinophilic angiocentric fibrosis
Synonyms: IgG4-related eosinophilic angiocentric fibrosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A rare otorhinolaryngologic disease characterized by an indolent submucosal mass of variable size and extent, most commonly arising in the anterior nasal cavity, involving the nasal septum and lateral nasal wall, and potentially extending into the adjacent sinuses. Occurrence in the larynx and lower respiratory tract or the orbit is rare. Histological examination shows concentric angiocentric stromal fibrosis (onionskin fibrosis) and prominent eosinophils. Increased numbers of IgG4-positive plasma cells in the lesion may also be observed, in addition to elevated serum IgG4. Patients typically present with long-standing obstructive symptoms.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Eosinophilic angiocentric fibrosis | IgG4-related eosinophilic angiocentric fibrosis |
| fr | Fibrose angiocentrique à éosinophiles | Fibrose angiocentrique à éosinophiles associée à IgG4 |
| de | Eosinophile angiozentrische Fibrose | Eosinophile angiozentrische Fibrose, IgG4-assoziierte |
| es | Fibrosis angiocéntrica eosinofílica | Fibrosis angiocéntrica eosinofílica asociada a IgG4 |
| it | Fibrosi angiocentrica eosinofila | Fibrosi angiocentrica eosinofila IgG4-correlata |
| nl | Eosinofiele angiocentrische fibrose | IgG4-gerelateerde eosinofiele angiocentrische fibrose |
| pt | Fibrose angiocêntrica eosinofílica | |
| pl | Eozynofilowe włóknienie angiocentryczne | Eozynofilowe włóknienie angiocentryczne związane z IgG4 |
| cs | Eosinofilní angiocentrická fibróza | IgG4 asociovaná eosinofilní angiocentrická fibróza |
| uk | Еозинофільний ангіоцентричний фіброз | Еозинофільний ангіоцентричний фіброз, повязаний з IgG4 |
| zh | 嗜酸性血管中心性纤维变性 | lgG4相关嗜酸性血管中心性纤维变性 |
| tr | Eozinofilik anjiyosentrik fibroz | IgG4-lişkili eozinofilik anjiyosentrik fibroz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J39.8 | NTBT | attributed | validated |
| ICD-11 | 4A43.0 | NTBT | index | validated |
| MONDO | 0018676 | E | validated | |
| UMLS | C5578050 | E | validated | |
| MedDRA | 10087310 | 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:284264IgG4-related disease
rare systemic and rheumatological diseases - ORPHA:98036Rare otorhinolaryngologic disease
rare otorhinolaryngological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 52 cases | Worldwide | Case(s) | Validated | 26674883[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_26674883[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/449566
- This record as JSON: /data/d/449566.json