Desquamative interstitial pneumonia
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare idiopathic interstitial pneumonia characterized by extensive, diffuse intra-alveolar accumulation of pigment-laden macrophages, most commonly associated with long-term exposure to tobacco smoke. Patients present with slowly progressive shortness of breath on exertion and chronic cough with bilateral crackles. Digital clubbing is also frequently observed. Pulmonary function test reveals a restrictive pattern. Computed tomography typically shows diffuse ground-glass opacities with subpleural and lower zone predominance.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Desquamative interstitial pneumonia | |
| fr | Pneumonie interstitielle desquamative | |
| de | Desquamative interstitielle Pneumonie | |
| es | Neumonía intersticial descamativa | |
| it | Polmonite desquamativa interstiziale | |
| nl | Desquamatieve interstitiële pneumonie | |
| pt | Pneumonia intersticial descamativa | |
| pl | Złuszczające śródmiąższowe zapalenie płuc | |
| cs | Deskvamační intersticiální pneumonie | |
| uk | Десквамативна інтерстиціальна пневмонія | |
| zh | 脱屑性间质性肺炎 | |
| tr | Deskuamatif interstisyel pnömoni |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| OMIM | 263000 | BTNT | validated | |
| ICD-10 | J84.1 | NTBT | attributed | validated |
| ICD-11 | CB03.3 | E | specific | validated |
| MONDO | 0009887 | E | validated | |
| UMLS | C0238378 | 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
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/98852
- This record as JSON: /data/d/98852.json