Idiopathic pleuroparenchymal fibroelastosis
Synonyms: IPPFE · Idiopathic pleuropulmonary fibroelastosis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Elderly
- Status
- active
A rare idiopathic interstitial pneumonia characterized by prominent subpleural and parenchymal fibroelastosis and pleural fibrosis, predominantly involving the upper lobes. Signs and symptoms include non-productive cough, dyspnea, and recurrent respiratory infections. Pneumothorax is a frequently reported complication. Pulmonary function test reveals a restrictive pattern and reduced diffusing capacity. Computed tomography shows pleural thickening with signs of fibrosis (traction bronchiectasis, architectural distortion, and loss of volume), and reticulation.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic pleuroparenchymal fibroelastosis | IPPFE · Idiopathic pleuropulmonary fibroelastosis |
| fr | Fibroélastose pleuroparenchymateuse idiopathique | Fibroélastose pleuropulmonaire idiopathique · IPPFE |
| de | Pleuroparenchymale Fibroelastose, idiopathische | Fibroelastose, idiopathische pleuropulmonare · IPPFE |
| es | Fibroelastosis pleuroparenquimatosa idiopática | FEPPI · Fibroelastosis pleuropulmonar idiopática |
| it | Fibroelastosi pleuroparenchimale idiopatica | |
| nl | Idiopathische pleuroparenchymale fibro-elastose | IPPFE |
| pt | Fibroelastose pleuroparenquimatosa idiopática | |
| pl | Samoistna fibroelastoza opłucnowo-śródmiąższowa | Idiopatyczna fibroelastoza opłucnej i miąższu płuca |
| cs | Idiopatická pleuroparenchymální fibroelastóza | IPPFE |
| uk | Ідіопатичний плевропаренхіматозний фіброеластоз | Ідіопатичний плевропульмональний фіброеластоз · IPPFE |
| zh | 特发性胸膜实质纤维弹性组织增生 | Idiopathic pleuropulmonary fibroelastosis |
| tr | İdiyopatik plöroparenkimal fibroelastoz | IPPFE · İdiyopatik plöropulmoner fibroelastoz |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J84.1 | NTBT | attributed | validated |
| ICD-11 | CB03.Y | NTBT | attributed | validated |
| MONDO | 0044633 | E | validated | |
| UMLS | C5567467 | 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:98300Idiopathic interstitial 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 |
|---|---|---|---|---|---|---|
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | ORPHANET_27141432[PMID]_28344924[PMID] |
| Cases/families | 37 cases | Worldwide | Case(s) | Validated | 27141432[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/494428
- This record as JSON: /data/d/494428.json