Isolated pulmonary capillaritis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Childhood
- Status
- active
Isolated pauciimmune pulmonary capillaritis is a small vessel vasculitis restricted to the lungs that may induce diffuse alveolar hemorrhage with dyspnea, anemia, chest pain, hemoptysis, bilateral and diffuse alveolar infiltrates at chest X-rays, without any underlying systemic disease. ANCA are frequently positive but could be negative.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Isolated pulmonary capillaritis | |
| fr | Capillarite pulmonaire isolée | |
| de | Lungen-Kapillaritis, isolierte | |
| es | Capilaritis pulmonar aislada | |
| it | Capillarite polmonare isolata | |
| nl | Geïsoleerde pulmonale capillaritis | |
| pt | Capilarite pulmonar isolada | |
| pl | Izolowane zapalenie naczyń włosowatych w płucach | |
| cs | Izolovaná plicní kapilaritida | |
| uk | Ізольований пульмонарний капілярит | |
| zh | 孤立性肺毛细血管炎 | |
| tr | İzole pulmoner kapillerit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | J84.0 | NTBT | attributed | validated |
| ICD-11 | CB04.4 | NTBT | index | validated |
| MONDO | 0017018 | E | validated | |
| UMLS | C3873357 | 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:264683Primary interstitial lung disease specific to childhood due to alveolar vascular disorder
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
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/264691
- This record as JSON: /data/d/264691.json