raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:52759

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.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enVasculitisSystemic vasculitis
frVasculariteVascularite systémique
deVaskulitisVaskulitis, systemische
esVasculitis
itVasculite
nlVasculitisBloedvatontsteking · Systemische vasculitis
ptVasculite
plZapalenie naczyńSystemowe zapalenie naczyń
csVaskulitidaSystémová vaskulitida
ukВаскулітСистемний васкуліт
zh血管炎系统性血管炎
trVaskülitSistemik vaskülit

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-114A44Especificvalidated
MONDO0018882Evalidated
MONDO18882Evalidated
MeSHD014657Evalidated
UMLSC0042384Evalidated
MedDRA10047115Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

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Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (2):

Children (5):

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Genes

0 direct

Orphanet records no gene directly on this code.

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Prevalence and incidence

1 record
TypeClassValueAreaQualificationValidationSource
Point prevalence1-9 / 100 0006.3 / 100 000EuropeValue and classNot yet validatedORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

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HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

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Sources for this card