Hypersensitivity pneumonitis
Synonyms: Extrinsic allergic alveolitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare, immunologically mediated, interstitial lung disease characterized by respiratory symptoms (cough, dyspnea) due to sensitization and subsequent hypersensitivity to environmental antigens. The disorder may be accompanied by systemic manifestations (weight loss, fatigue).
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Hypersensitivity pneumonitis | Extrinsic allergic alveolitis |
| fr | Alvéolite allergique extrinsèque | AAE · Pneumopathie d'hypersensibilité |
| de | Exogen-allergische Alveolitis | Hypersensitivitätspneumonie |
| es | Neumonitis por hipersensibilidad | Alveolitis alérgica extrínseca |
| it | Polmonite da ipersensibilità | Alveolite allergica estrinseca · HP |
| nl | Hypersensitiviteitspneumonitis | Extrinsieke allergische alveolitis · Overgevoeligheidspneumonitis |
| pt | Pneumonite de hipersensibilidade | Alveolite alérgica extrínseca |
| pl | Alergiczne zapalenie pęcherzyków płucnych | Extrinsic allergic alveolitis · HP |
| cs | Hypersenzitivní pneumonitida | HP |
| uk | Гіперчутливий пневмоніт | Зовнішній алергічний альвеоліт |
| zh | 过敏性肺炎 | 外源性过敏性肺泡炎 |
| tr | Hipersensitif pnömoni | Ekstrinsik alerjik alveolit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 145300 | BTNT | validated | |
| ICD-10 | J67.8 | NTBT | attributed | not yet validated |
| ICD-11 | CA70 | E | specific | validated |
| MONDO | 0017853 | E | validated | |
| MeSH | D000542 | E | validated | |
| UMLS | C0002390 | E | validated | |
| GARD | 12 | E | validated | |
| MedDRA | 10081988 | 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:264984Exposure-related interstitial lung disease
rare respiratory diseases; rare transplant-related disorders - ORPHA:98052Rare allergic respiratory disease
rare allergic disease
Codes that Orphanet moved or referred to this one:
- ORPHA:99906OBSOLETE: Farmer's lung disease referred to · inactive
- ORPHA:99907OBSOLETE: House allergic alveolitis referred to · inactive
- ORPHA:99908OBSOLETE: Pigeon-breeder lung disease referred to · inactive
- ORPHA:99909OBSOLETE: Occupational allergic alveolitis referred to · inactive
Genes
0 direct · 1 via subtypesOrphanet records no gene directly on this code.
Recorded by Orphanet on subtypes of this disorder:
| Gene | Subtype | Association | Status | HGNC | Ensembl | OMIM | Locus | Name | Source of validation |
|---|---|---|---|---|---|---|---|---|---|
| MUC5B | ORPHA:686465Fibrotic hypersensitivity pneumonitis | Major susceptibility factor in | Assessed | HGNC:7516 | ENSG00000117983 | 600770 | 11p15.5 | mucin 5B, oligomeric mucus/gel-forming | 28648751[PMID] |
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
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/31740
- This record as JSON: /data/d/31740.json