Idiopathic acute eosinophilic pneumonia
Synonyms: IAEP
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- All ages
- Status
- active
Idiopathic acute eosinophilic pneumonia (IAEP) is an eosinophilic pneumonia of undetermined etiology that is characterized by acute febrile hypoxic respiratory failure associated with diffuse radiographic infiltrates and pulmonary eosinophilia, but without concurring allergy or infection.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic acute eosinophilic pneumonia | IAEP |
| fr | Pneumonie à éosinophiles idiopathique aiguë | IAEP · Syndrome de Loeffler · Syndrome de Loffler |
| de | Pneumonie, eosinophile idiopathische, akute Form | = en |
| es | Neumonía eosinofílica idiopática aguda | = en |
| it | Polmonite eosinofila acuta idiopatica | = en |
| nl | Idiopathische acute eosinofiele pneumonie | = en |
| pt | Pneumonia eosinofílica aguda idiopática | |
| pl | Idiopatyczne ostre eozynofilowe zapalenie płuc | = en |
| cs | Idiopatická akutní eozinofilní pneumonie | IAEP · Loefflerův syndrom · Löfflerův syndrom |
| uk | Ідіопатична гостра еозинофільна пневмонія | = en |
| zh | 特发性急性嗜酸粒细胞肺炎 | = en |
| tr | İdiyopatik akut eozinofilik pnömoni | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J82 | NTBT | inclusion | validated |
| ICD-11 | CB02.10 | E | specific | validated |
| MONDO | 0019122 | E | validated | |
| UMLS | C4518469 | E | validated | |
| GARD | 519 | 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 (1):
- ORPHA:182101Idiopathic eosinophilic pneumonia
rare respiratory diseases; rare transplant-related disorders
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 | 100 cases | Worldwide | Case(s) | Validated | 12403693[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | 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
11 annotations · validated 2016-06-01| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0100749 | Chest pain | |
| HP:0001945 | Fever | |
| HP:0002113 | Pulmonary infiltrates | |
| HP:0002093 | Respiratory insufficiency | |
| HP:0002091 | Restrictive ventilatory defect | |
| Frequent (79-30%) | ||
| HP:0002027 | Abdominal pain | |
| HP:0002793 | Abnormal pattern of respiration | |
| HP:0001879 | Abnormality of eosinophils | |
| HP:0002103 | Abnormality of the pleura | |
| HP:0012735 | Cough | |
| HP:0003326 | Myalgia | |
Run the differential on the 5 obligate / very frequent terms
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/724
- This record as JSON: /data/d/724.json