1. Aortitis Infectious Noninfectious -takayasu disease
-giant cell arteritis
-spondyloarthropathies
-behcet syndrome
-RA
-SLE
-relapsing polychondritis
-cogan syndrome
-sarcoidosis
-idiopathic retroperitoneal fibrosis

2. Takayasu Arteritis

3. Aortic arch syndrome
Pulseless disease

4. General consideration
Greatest frequency in Asian countries but distribution of disease is worldwide
Unknown etiology(auto immune mechanism?)
Women are affected about 10 times more often than men.
Median age of onset is 25 yrs

5. The incidence of TA is estimated to be 2
The incidence of TA is estimated to be 2 .6 per 1,000,000 persons in the United States and per 1,000,000 in northern Europe . Autopsy series from Japan point to a higher incidence, with 1 in every 3000 autopsies having a features of TA .

6. pathogenesis Granulomatous lesion similar to giant cell arteritis.
Mononuclear cell infilteration
macrophages
lymphocyte ( T,gamma-delta,cytotoxic,natural killer)
B lymphocyte
Cytokaines (IL-6,TNF)

7. Histo pathology Active stage ……..granulomatous arteritis
Late stage…………. Medial degeneration,fibrous scaring,intimal proliferation,thrombosis

10. Aneurysm formation is less common than stenosis(3-4 times)
An important cause of death is aneurysm rupture
Left sub clavian artery involvement….90%
Thoracic aortic lesion…..66%
Abdominal aorta……..50%
Aortoiliac…….12%
Pulmonary arteritis………50%

11. Symptoms ,signs,lab tests
Pre pulseless phase (active )
fever
night sweat
malaise
nausea ,vomiting
weight loss
rash
arthralgia
Raynaud phenomenon
splenomegaly
acceleration of ESR
elevated CRP
anemia
plasma protein abnormalities

12. Hypertension is most often due to renal artery stenosis but can also be associated with suprarenal aortic stenosis or a chronically damaged, rigid aorta

13. Chronic phase Upper exteritities claudication Stroke TIA Dizziness
Syncope
Retinopathy
HTN….. ›50%
Angina pectoris MI HF
Pericarditis(infrequent)
Intermittent claudicasion of lower limb

14. prognosis Ischemic event or Deth over 5 yr……25-30%
HTN ,cardiac involvement are associated with shortened life expectancy.

15. Diagnosis Major criteria 1- onset of illness‹ 40 yr
2- upper exterimity claudication
3- diminished brachial pulse
4- ›10 mmhg difference systolic
BP in the arms
5 -Subclavian or aortic bruit
6- narrowing of the aorta or
major branches
Presence of three criteria ……..high diagnostic accuracy

16. Management
Active phase………….corticosteroid and immunosuppressive therapy,cytotoxic agents
Arterial stenosis ,obstruction……surgical operation,angioplasty and stenting