Respiratory bronchiolitis-interstitial lung disease syndrome
Synonyms: RB-ILD
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
Respiratory bronchiolitis - interstitial lung disease is a mild inflammatory pulmonary disorder developed by cigarette smokers and characterized by shortness of breath and cough, pulmonary function abnormalities of mixed restrictive and obstructive lung disease and high resolution CT scanning showing centrilobular micronodules, ground glass opacities and peribronchiolar thickening.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Respiratory bronchiolitis-interstitial lung disease syndrome | RB-ILD |
| fr | Bronchiolite respiratoire avec pneumopathie interstitielle | |
| de | Respiratorische Bronchiolitis mit interstitieller Lungenerkrankung | = en |
| es | Síndrome de bronquiolitis respiratoria-enfermedad pulmonar intersticial | = en |
| it | Bronchiolite respiratoria - pneumopatia interstiziale | = en |
| nl | Respiratoire bronchiolitis - interstitiële longziekte-syndroom | = en |
| pt | Síndrome de bronquiolite respiratória-doença pulmonar intersticial | = en |
| pl | Zapalenie oskrzelików - śródmiąższowa choroba płuc | = en |
| cs | Syndrom zahrnující respirační bronchiolitidu a intersticiální plicní chorobu | = en |
| uk | Синдром респіраторного бронхіоліту-інтерстиціального захворювання легень | = en |
| zh | 呼吸性细支气管炎-间质性肺疾病综合征 | = en |
| tr | Solunum bronşiyolit-interstisyel akciğer hastalığı sendromu | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J68.4 | NTBT | attributed | validated |
| ICD-11 | CB03.6 | E | specific | validated |
| MONDO | 0019204 | E | validated | |
| UMLS | C1735355 | E | validated | |
| MedDRA | 10066393 | 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
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Not yet 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
22 annotations · validated 2021-09-28| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0006510 | Chronic pulmonary obstruction | |
| HP:0002207 | Diffuse reticular or finely nodular infiltrations | |
| HP:0002094 | Dyspnea | |
| HP:0002097 | Emphysema | |
| HP:0025179 | Ground-glass opacification on pulmonary HRCT | |
| HP:0031996 | Inspiratory crackles | |
| HP:0030877 | Reduced FEV1/FVC ratio | |
| HP:0002091 | Restrictive ventilatory defect | |
| HP:0030828 | Wheezing | |
| Occasional (29-5%) | ||
| HP:0004469 | Chronic bronchitis | |
| HP:0045051 | Decreased DLCO | |
| HP:0006516 | Hypersensitivity pneumonitis | |
| HP:0012418 | Hypoxemia | |
| HP:0031246 | Nonproductive cough | |
| HP:0011947 | Respiratory tract infection | |
| Very rare (<4-1%) | ||
| HP:0002110 | Bronchiectasis | |
| HP:0100749 | Chest pain | |
| HP:0001217 | Clubbing | |
| HP:0001945 | Fever | |
| HP:0002105 | Hemoptysis | |
| HP:0025175 | Honeycomb lung | |
| HP:0001824 | Weight loss | |
Annotation source: 25011486[PMID]_32551095[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/79127
- This record as JSON: /data/d/79127.json