Takayasu arteritis

Takayasu arteritis


INTRODUCTION:

  • It is granulomatous vasculitis affecting medium and larger arteries.
  • The disease has a strong predeliction for the aortic arch and its branches. C.) Takayasus arteritis classically involves the “aortic arch”.

Histological changes

Transmural involvement (all three layers are involved).

Media  →  Mononuclear infiltrates to granulomatous inflammation with giant cells

Adventitia → Mononuclear infiltrates

Vasa vasorum → Perivascular cuffing

  • As the disease progresses collagenous scarring with admixed chronic infiltrates occur in all three layers of the vessel wall.
  • Narrowing of the lumen occurs with or without thrombosis pathological changes in various organs reflect the compromise of blood flow through the involved vessels.
  • Aortic lesions of Takayasu arteritis share many attributes with Giant cell arteritis including clinicalfeatures and histology.

The distinction is typically made on the basis of the age of the patient

> 50 years  → Giant cell Arteritis

< 50 yrs → Takayasu disease

Clinical features of Takayasu’s Arteritis 😕

Nonspecific features of Vasculitis

  • Fatigue, weight loss and fever
  • Vascular symptoms (Due to luminal narrowing of the aortic arch).
  • Marked weakening of pulses in upper extremities It is also knowns as “pulseless disease”.
  • Reduced blood pressure in upper extremitiese
  • Ocular disturbances
  • Visual defects
  • Retinal hemorrhages
  • Total blindness
  • Takayasu disease can also cause Myocardial infarction.
  • Due to narrowing of the coronary arteries.
  • Hypertension in 50% cases
  • Narrowing of renal arteries lead to hypertension in half the cases.
  • Diminished pulsein upper extremities and hypertension in a young female suggests a diagnosis of Takayasu arteritis.

Criteria

Definition

Age at disease onset in year

Development of symptoms of findings related to Takayasu arteritis at age < 40 years

Claudication of examination

Development and worsening of fatigue and discomfort in muscles of one or more extremity while in use, especially the upper extremities.

 Decreased brachial artery pulse  Decreased pulsation of one or both brachial arteries

BP differences> 10 mmHg

 Difference of> 10 mmHg in systolic blood pressure between arms

Bruitover subclavian arteries or aorta

Bruit audible on auscultation over one or both subclavian arteries or abdominal aorta

Arteriogram abnormality

 

Arteriographic narrowing or occlusion of the entire aorta, its primary branches, or large arteries in the proximal upper or lower extremities, not due arteriosclerosis, fibro-muscular dysplasia, or similar causes: changes usually focal or segmental 

Takayasu arteritis is diagnosed if at least three of six criteria are present.

The most commonly affected arteries as seen by arteriography in order of frequency

  • Coronary (<10%)
  • Vertebral (35%)
  • Coeliac axis (18%)
  • Pulmonary (10-40 %)
  • Superior Mesenteric (18%)
  • Iliac (17%)
  • Subclavian (93%)
  • Common Carotid (58%)
  • Abdominal Aorta (47%)
  • Renal (38%)

Exam Important

  • Most common causeof renal artery stenosis in children in India is Takayasu Aortoarteritis
  • Takayasu arteritis commonly effects Renal arteries
  • Takayasu arteritis  are associated with granulomatous pathology
  • Most common cause of renovascular hypertension in children in India is Takayasu Aortoarteritis
  • Aortic arch syndrome is also known as Takayasu Aortoarteritis
  • Takayasu Aortoarteritis is Large vessel vasculitis 
  • Takayasu Aortoarteritis is Pulseless disease 
  • Takayasu’s arteritis predispoase to Aortic dissection
  • A young female presents with diminished pulses in the upper limb and hypertension. The most likely diagnosis is Takayasu’s arteritis
  • Reversed Coarctation is seen in Takayasu Arteritis
  • Takayasu arteritis mainly affects Subclavian artery
  • Renal artery stenosis may occur in Takayasu arteritis
  • Blood pressure difference between left and right limbs in Takayasu arteritis
  • Takayasu arteritis Shows Strongly positive mantoux
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