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Takayasu arteritis

Orpha number ORPHA3287
Synonym(s) -
Prevalence 1-9 / 1 000 000
Inheritance
  • Sporadic
Age of onset Adulthood
ICD-10
  • M31.4
OMIM
UMLS
  • C0039263
MeSH
  • D013625
MedDRA
  • 10043097
SNOMED CT
  • 239937004
  • 359789008

Summary

Takayasu arteritis is an inflammatory arteritis affecting large vessels, predominantly the aorta and its main branches. Thickening of the vessel wall is an early hallmark of the disease and leads to stenosis, thrombosis, and sometimes aneurysm formation. The manifestations are highly polymorphic, patients can be either asymptomatic or present with severe neurological symptoms. The reported incidence ranges from 1.2 to 2.6/million/year. Women between the ages of 20 and 40 are more frequently affected than men. The etiology of the disease is unknown but several hypotheses, including those suggesting an infectious or autoimmune origin for the syndrome, have been proposed. Diagnosis is based on imaging methods. Doppler ultrasound, computed tomography, and magnetic resonance imaging are fast and reliable methods for assessing vessel anatomy and luminal status. Positron emission tomography with fluorodeoxyglucose appears to be a highly sensitive and effective method for detecting disease activity, especially since standard inflammatory markers seem ineffective. Until now, corticosteroids have been the treatment of choice. If remission does not occur, methotrexate is added. Percutaneous transluminal angioplasty and sometimes vascular surgery is necessary in cases of critical ischemia or threatening aneurysm. Duration of treatment, choice of second-line treatment, and protocol for tapering medication, currently depend more on experience than on evidence-based medicine. Multicenter studies are needed to guide future practice. The prognosis for patients mainly depends on the occurrence of complications such as retinopathy, arterial hypertension, aneurisms and aortic insufficiency.

Expert reviewer(s)

  • Pr Eric HACHULLA
  • Dr Thomas QUEMENEUR

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Detailed information

Summary information
Review article
  • FR (2006,pdf)
Article for general public
  • FR (2006,pdf)
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