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Aortitis

Aortitis. Infectious Noninfectious - takayasu disease -giant cell arteritis - spondyloarthropathies - behcet syndrome -RA -SLE

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Aortitis

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

  2. TakayasuArteritis

  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 .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 .

  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 ……..granulomatousarteritis Late stage…………. Medial degeneration,fibrousscaring,intimalproliferation,thrombosis

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

  9. 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

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

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

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

  13. Diagnosis • Major criteria 1- onset of illness‹ 40 yr 2- upper exterimityclaudication 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

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

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