ORPHA:1164
Allergic bronchopulmonary aspergillosis
Synonyms: ABPA · Allergic aspergillosis · Hinson-Pepys disease
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
A rare immunologic pulmonary disorder caused by hypersensitivity to <i>Aspergillus fumigatus</i>, clinically manifesting with poorly controlled asthma and recurrent pulmonary infiltrates.
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Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Allergic bronchopulmonary aspergillosis | ABPA · Allergic aspergillosis · Hinson-Pepys disease |
| fr | Aspergillose broncho-pulmonaire allergique | Maladie de Hinson-Pepys · ABPA · Aspergillose allergique |
| de | Aspergillose, allergische bronchopulmonale | ABPA · Hinson-Pepys-Krankheit · Lungenaspergillose, allergische |
| es | Aspergilosis bronco-pulmonar alérgica | ABPA · Aspergilosis alérgica · Enfermedad de Hinson-Pepys |
| it | Aspergillosi broncopolmonare allergica | ABPA · Aspergillosi allergica · Malattia di Hinson-Pepsy |
| nl | Allergische bronchopulmonale aspergillose | ABPA · Allergische aspergillose · Ziekte van Hinson-Pepys |
| pt | Aspergilose broncopulmonar alérgica | Doença de Hinson-Pepys |
| pl | Alergiczna aspegiloza oskrzelowo-płucna | ABPA · Aspergiloza alergiczna · Choroba Hinsona i Pepysa |
| cs | Alergická bronchopulmonální aspergilóza | ABPA · Alergická aspergilóza · Hinsonova-Pepysova nemoc · Hinson-Pepysova nemoc |
| uk | Алергічний бронхолегеневий аспергільоз | ABPA · Алергічний аспергільоз · Хвороба Хінсона-Пепіса |
| zh | 变应性支气管肺曲霉菌病 | 变应性曲霉菌病 |
| tr | Alerjik bronkopulmoner aspergilloz | ABPA · Hinson-Pepys hastalığı · Alerjik aspergilloz |
Definition in:
2
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 103920 | BTNT | validated | |
| ICD-10 | B44.1+ | NTBT | attributed | validated |
| ICD-10 | J99.8* | NTBT | attributed | validated |
| ICD-11 | CA82.4 | NTBT | index | validated |
| MONDO | 0015243 | E | validated | |
| MeSH | D001229 | E | validated | |
| UMLS | C0004031 | E | validated | |
| GARD | 602 | E | validated | |
| MedDRA | 10006474 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
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Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (2):
- ORPHA:98052Rare allergic respiratory disease
rare allergic disease - ORPHA:101944Rare pulmonary disease
rare respiratory diseases
No children.
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Genes
0 directOrphanet records no gene directly on this code.
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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.
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HPO phenotypes
14 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0001879 | Abnormality of eosinophils | |
| HP:0002715 | Abnormality of the immune system | |
| HP:0002099 | Asthma | |
| HP:0012735 | Cough | |
| Frequent (79-30%) | ||
| HP:0002110 | Bronchiectasis | |
| HP:0002120 | Cerebral cortical atrophy | |
| HP:0011134 | Low-grade fever | |
| HP:0001824 | Weight loss | |
| Occasional (29-5%) | ||
| HP:0025426 | Abnormal bronchus morphology | |
| HP:0001231 | Abnormal fingernail morphology | |
| HP:0002097 | Emphysema | |
| HP:0002105 | Hemoptysis | |
| HP:0002092 | Pulmonary arterial hypertension | |
| HP:0002093 | Respiratory insufficiency | |
Run the differential on the 4 obligate / very frequent terms
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Sources for this card
- Orphanet page: orpha.net/en/disease/detail/1164
- This record as JSON: /data/d/1164.json