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

Takayasu Arteritis

Aortic arch syndrome Pulseless disease

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

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 1 .26 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 .

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)

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

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%

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

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

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

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

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

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