Takayasu’s Arteritis

Epidemiology

  • aka “Pulseless Disease”
  • First reported in 1908
  • Occurs mainly in Asian (mostly Japanese) females
  • Peak age: <40 y/o
  • Sex: F:M ratio 9 to 1

Physiology

  • granulomatous arteritis of medium and large-sized arteries
  • Arterial Involvement:
    • Aorta
    • Aortic branches: subclavian arteries are the most ocmmonly affected
    • Renal vessels
    • Large arteries of LE

Pathology

  • granulomatous vasculitis involving aorta and branches -> narrowing, occlusion, and aneurysm formation
  • Pulmonary arteritis probably occurs in most patients (pulmonary involvement can occur without other organ involvement)

Diagnosis

  • ABG: elevated A-a gradient
  • CBC: mild anemia/ leukocytosis
  • ESR: elevated
  • CRP: elevated
  • PFT’s: decreased DLCO/ increased Vd/Vt ratio
  • MR angiogram: may reveal changes (vessel wall thickening) before frank loss of pulses
  • Angiogram: usually diagnostic

ECHO:
– Pulm HTN: may mimic CTEPH


Clinical

  • Aortic arch: aneurysm
  • CNS: CVA (15% of cases)
  • Ocular: visual symptoms (60%)/ blindness
  • Pulmonary:
    • Pulmonary HTN: PA’s involved in most patients
  • Rheumatologic: arthralgias
  • Systemic: weight loss, fatigue, fever
  • Cardiovascular:
    • Hypertension (50%/due to renal artery involvement)
    • Syncope (50%)
    • Absent UE pulses (“pulseless disease”)
    • LV failure
    • Limb claudication
    • Bruits over large arteries
    • MI: although coronary arteries are rarely involved

Symptoms/signs: may occur with or without systemic vascular involvement
1) Optho:
a) Cataracts:
b) AVM’s around optic disks:
c) Blindness:
2) Cardiovascular:
a) Absence of pulses
b) Severe HTN:
c) LV failure:
d) Aortic aneurysm rupture
3) Neuro:
a) CVA
4) Pulmonary:
a) Pulmonary HTN: may mimic CTEPH (as pulmonary involvement can occur without other organ involvement in some cases)


Treatment

  • Corticosteroids: disease may progress with blindness, despite treatment
  • Cytoxan: for severe cases
  • Mycophenolate: may be effective as steroid-sparing agent
  • Methotrexate: may be effective as steroid-sparing agent
  • Anticoagulation

References

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