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Vasculitis

Orpha number ORPHA52759
Synonym(s) Systemic vasculitis
Prevalence 1-9 / 100 000
Inheritance -
Age of onset -
ICD-10 -
OMIM -
UMLS
  • C0042384
  • C0264939
MeSH
  • D014657
  • D056647
MedDRA
  • 10036023
  • 10047115
SNOMED CT
  • 31996006
  • 46956008

Summary

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; see these terms), medium-sized vessels (medium-vessel vasculitis e.g. polyarteritis nodosa and Kawasaki disease; see these terms), or small-sized vessels (small-vessel vasculitis, e.g. Wegener granulomatosis, microscopic polyangiitis, immunoglobulin A vasculitis, and cutaneous leukocytoclastic angiitis; see these terms). Vasculitis occurs at any age, may be acute or chronic, and manifest 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, going from life or sight threatening fulminant disease (e.g. Behçet disease) to relatively minor skin disease.


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