Takayasu

1
Takayasus Disease

• Arteritis affecting primarily the aorta and its
  main branches
• Leads to segmental stenosis, occlusion,
  dilatation, and aneurysm formation
• Pathology shows intimal proliferation and
  fibrotic contraction of media and adventitia as
  well as granulomatous vasculitis
• Initially thought to affect only young Asian
  women in arch vessels
• First described in 1908 with regard to retinal
  central vessel changes
• Occurs more frequently in the Far East
• 1/3000 autopsy cases in Japan
• 2.6 cases/1 million incidence in U.S.
• 6.4 cases/1 million incidence in Sweden

2
Etiology

• UNKNOWN!
• Known association with microorganisms and
  aortitis
• Reports with Tb (60 of autopsy cases)
• HLA associations
• Tendency to affect women of reproductive age
• Autoimmune influences
• Common association with IBD, SLE, PMR, AS, RA
• Elevated gamma globulins, RF

3
Clinical Features

• Females affected 7-8x more frequently
• Occurs in patients younger than 40
• Great Imitator
• Phases of disease process
• Early (systemic inflammatory)
• Fever, myalgias, arthralgias, weight loss,
  carotidynia
• HTN, vascular bruits, asymmetric arm BP, early
  ischemic symptoms
• Late (occlusive)
• Ocular signs
• HTN (renal artery stenosis or aortic coarctation)
• Aortic insufficiency
• CHF/CAD
• Associated with cutaneous changes
• Erythema nodosum pyoderma gangrenosum

4
Classification

• Divided into subtypes based on location
• Type V most common (60-70)

5
Diagnosis

• Based on clinical features and imaging studies
  obtained at routine intervals
• Duplex imaging
• Screen for renal, mesenteric, carotid,
  subclavian, and axillary abnormalities
• Angiography
• Narrowing of aorta or other major branches
• Can be short and segmental, or long and diffuse
• Fusiform or saccular aneurysms

6
(No Transcript)
7
(No Transcript)
8
(No Transcript)
9
Therapy

• Relieve systemic manifestations and treat
  inflammatory process on vessels
• Prednisone 1mg/kg/day for 1-3 mo, with a 6-12mo
  taper
• Cyclophosphamide
• methotrexate
• Identify and treat complications of the vascular
  disease
• PTA of renal and iliac vessels
• Timing is controversial, as is use of stents
• Restenosis rates 15-20

10
Surgical Therapy

• Cerebrovascular disease
• Stroke, TIA, amarosis occur in 8-35
• Bypass recommended from ascending aorta
• Renovascular disease
• HTN present in 20-72
• PTA, then consider renovascular reconstruction
• Mesenteric disease
• Incidence of involvement ranges from 5-66
• Bypass for symptomatic patients
• Aneurysms
• Incidence of aneurysm in Takayasus ranges from
  22-32
• Can be multiple, saccular or fusiform, associated
  with stenotic lesions
• Most commonly found in ascending aorta, thoracic,
  or abdominal aorta
• Incidence of aneurysm rupture is low

11
Outcomes

• 50 later experience relapse of initial symptoms
• 94 5 year survival
• Survival after surgery can be up to 20-30 years
• Low likelihood of anastamotic problems
• Death usually related to vascular complications
  from HTN, AI, stroke