Vasculitis
Synonyms: Systemic vasculitis
- Type
- Category
- Group
- Group of disorders
- Status
- active
Vasculitis represents a clinically heterogenous group of diseases of multifactorial etiology characterized by inflammation of either large-sized vessels (large-vessel vasculitis, e.g. Giant-cell arteritis and Takayasu arteritis), medium-sized vessels (medium-vessel vasculitis e.g. polyarteritis nodosa and Kawasaki disease), or small-sized vessels (small-vessel vasculitis, e.g. granulomatosis with polyangiitis, microscopic polyangiitis, immunoglobulin A vasculitis, and cutaneous leukocytoclastic angiitis). Vasculitis occurs at any age, may be acute or chronic, and manifests with general symptoms such as fever, weight loss and fatigue, as well as more specific clinical signs depending on the type of vessels and organs affected. The degree of severity is variable, ranging from life or sight threatening disease (e.g. Behçet disease) to relatively minor skin disease.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Vasculitis | Systemic vasculitis |
| fr | Vascularite | Vascularite systémique |
| de | Vaskulitis | Vaskulitis, systemische |
| es | Vasculitis | |
| it | Vasculite | |
| nl | Vasculitis | Bloedvatontsteking · Systemische vasculitis |
| pt | Vasculite | |
| pl | Zapalenie naczyń | Systemowe zapalenie naczyń |
| cs | Vaskulitida | Systémová vaskulitida |
| uk | Васкуліт | Системний васкуліт |
| zh | 血管炎 | 系统性血管炎 |
| tr | Vaskülit | Sistemik vaskülit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-11 | 4A44 | E | specific | validated |
| MONDO | 0018882 | E | validated | |
| MONDO | 18882 | E | validated | |
| MeSH | D014657 | E | validated | |
| UMLS | C0042384 | E | validated | |
| MedDRA | 10047115 | 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 (2):
- ORPHA:98023Rare systemic or rheumatologic disease
rare systemic and rheumatological diseases - ORPHA:68362Rare vascular disease
rare circulatory system diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
1 record| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 6.3 / 100 000 | Europe | Value and class | 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/52759
- This record as JSON: /data/d/52759.json