TAKAYASU
Video presentation
Epidemiology | |||||
---|---|---|---|---|---|
Gender ratio |
1:1 |
Average onset age |
20-40y |
Incidence |
2/106 |
Disease presentation
The disease is a systemic vasculitis affecting large/medium sized blood vessels. Its diagnosis mainly relies on imaging of arterial lesions.
Main clinical symptoms
Fever as an early phase sign with excessive fatigue, involuntary weight loss, general weakness
Vessel inflammation (and poor circulation): inflamed aorta and subsequent arteries leading to coldness, cyanosis, pain in hands and feet, headache, claudication
Biological signs
Increase in C-reactive protein (CRP) for 50% of patients
Absence of AntiNeutrophil Cytoplasmic Antibodies (ANCA)
Therapy
Corticosteroids combined with immunomodulating drugs (methotrexate, azathioprine, anti-TNFα, anti-IL6)