Non-specific interstitial pneumonia
Synonyms: NSIP · Non-specific idiopathic interstitial pneumonia
- Type
- Disease
- Group
- Disorder
- Status
- active
A rare idiopathic interstitial pneumonia characterized by temporally uniform alveolar and interstitial mononuclear cell inflammation (cellular type) and/or fibrosis of the alveolar walls (fibrotic type) with preserved alveolar architecture. Other types of interstitial lung disease must be excluded. Symptoms are non-specific and include dyspnea, cough, and often constitutional symptoms such as fever and fatigue. Pulmonary function test reveals a restrictive pattern. Computed tomography shows predominantly lower lobe subpleural reticular changes, traction bronchiectasis, and ground-glass opacities. The cellular type of the disease is less common but carries a better prognosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Non-specific interstitial pneumonia | NSIP · Non-specific idiopathic interstitial pneumonia |
| fr | Pneumonie interstitielle non spécifique | NSIP · Pneumonie interstitielle idiopathique non spécifique |
| de | Pneumonie, interstitielle, unspezifische | NSIP · Pneumonie, interstitielle, idiopathische, unspezifische |
| es | Neumonía intersticial no específica | NSIP · Neumonía intersticial no específica idiopática |
| it | Polmonite interstiziale aspecifica | NSIP · Polmonite interstiziale idiopatica non specifica |
| nl | Niet-specifieke interstitiële pneumonie | NSIP · Niet-specifieke idiopathische interstitiële pneumonie |
| pt | Pneumonia intersticial não-específica | |
| pl | Niespecyficzne śródmiąższowe zapalenie płuc | Niespecyficzne idiopatyczne śródmiąższowe zapalenie płuc · NSIP |
| cs | Nespecifická intersticiální pneumonie | NSIP · Nespecifická idiopatická intersticiální pneumonie |
| uk | Неспецифічна інтерстиціальна пневмонія | Неспецифічна ідіопатична інтерстиціальна пневмонія · NSIP |
| zh | 非特异性间质性肺炎 | 非特异性特发性间质性肺炎 |
| tr | Spesifik olmayan interstisyel pnömoni | NSIP · Spesifik olmayan idiyopatik interstisyel pnömoni |
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 | index | validated |
| MONDO | 0019622 | E | validated | |
| UMLS | C1290344 | 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/91364
- This record as JSON: /data/d/91364.json