Takayasu’s Aortitis and Arteritis

The Common Vein Copyright 2012

Jameel Tariq Galloway Ashley Davidoff MD

 

Definition

Takayasu’s arteritis is an inflammatory disease of the aorta and its first order branches

It is caused by intimal proliferation and fibrosis along with fibrous scarring and degeneration of the elastic fibers of the media of the aorta and large arteries.  The M:F is 1:8.  Typical age of onset is in teenage years.

• As a result of the inflammatory process, –
  •  vaso vasorum are destroyed
  • necrotizing and obliterative segmental,
  • panarteritis
    • the adventitia becomes thickened and the
  • large-vessel
  • late complications
    • Localized aneurysm formation,
    • post-stenotic dilatation and
    • calcification in the arterial walls are .  The process most often involves the arch and its major branches

The clinical diagnosis is suspected when a teenage patient presents with loss of pulses or ischemic parestesias. Non specific findings include  fever, malaise, night sweats, arthralgias, fatigue and occasionally pain and tenderness over the affected arteries. Imaging is best accomplished with CTA or MRA and for the smaller vessels angiography is still a useful modality.

“Aortic wall thickening, which has been described as a “double ring” appearance at contrast-enhanced CT, is the typical finding in the early stage, with a poorly enhanced internal ring (the swollen intima) and an enhancing outer ring (the inflamed media and adventitia) (9).” (Restrepo)

 

Treatment options include glucocorticoids may relieve systemic symptoms and surgical treatments may be needed for late complications Morbidity and mortality depend on the presence or absence of severe complications such as retinopathy, aortic regurgitation, secondary hypertension or aortic aneurysms There is 97% survival over 7 years in uncomplicated disease and 59% in patients with complications

Smooth Narrowing – Takayasu’s Aortitis 20354b01 14 year old male artery thoracic aorta fx smooth narrowing of isthmus of aorta Takayasu’s aortitis angiography angiogram Courtesy Ashley Davidoff MD Multicentric and Diffuse Irregular Narrowing of the Istmus and Abdominal Aorta Takayasu’s Arteritis In this patient, the MRI shows narrowing of the abdominal aorta segmentally and diffusely in this patient who has Takayasu’s arteritis and aortitis – an inflammatory condition affecting the aorta and large arteries.  16917b Courtesy Ashley Davidoff MD Takayasu’s Arterirtis The series of images are from the angiogram of a 14 year old female who presented with seizures and an elevated blood pressure. Images a and b show multiple stenoses within the carotids best seen at the level of the bifurcation into external and internal arteries. In addition in b, the aortic arch shows non critical narrowing just after the origin of the left common carotid vessel. Note that the right subclavian artery is not seen and presumably is accluded at its origin. The abdominal angiogram shows a significant narrowing of the left renal artery with post stenotic dilitation, and stenotic disease in the infrarenal abdominal aorta. The multicentric nature of the disease in a young female is athognomonic of Takayasu’s arteritis. 35155c Courtesy of Laura Feldman MD. code CVS artery aorta arteritis inflammation Takayasu’s carotid thorax arch renal abdomen pulseless —

 

References

“Takayasu Arteritis.” Medline Plus. National Library of Medicine, n.d. Web. 14 June 2012. <http://www.nlm.nih.gov/medlineplus/ency/article/001250.htm>.