TAKAYASU'S ARTERITIS

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DEFINITION
Vasculitis of medium / large vessels.
See vasculitis card for place in schema.
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INCIDENCE
Uncommon, much less than temporal arteritis.
Sex
Adolescent girls and young women especially.
Geography
More common in the orient.
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AETIOLOGY
Refer vasculitis.
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BIOLOGICAL BEHAVIOUR
Pathophysiology
Affects medium and large-sized arteries.
Strong predilection for the aorta and its branches.
Pulmonary may also be involved.
Symptoms depend on the location of the involved vessel.

Pathology
Panarteritis with mononuclear cells and occasionally giant cells.
Degeneration of the elastic lamina.
Narrowing of the lumen with or without thrombosis.
Vasa vasorum frequently involved.

Natural History
Course may be fulminant, may progress gradually, or may stabilise.
Complications relate to the distribution of the involved vessels.
Death usually occurs from CHD or vertebral manifestations.
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MANIFESTATIONS

Symptoms/Signs
Local
By vessel:
Subclavian: Arm claudication, Raynaud's.
Common carotid: Visual changes, syncope, TIAs, stroke.
Abdominal aorta: Abdo pain, nausea, vomiting.
Renal: Hypertension, renal failure.
Upper aorta: Aortic regurge, CHD.
Vertebral: Visual changes, dizziness.
Gut: Abdo pain nausea, vomiting.
Iliac: Leg claudication.
Pulmonary: Atypical chest pain.
Coronary: Chest pain, MI.

Systemic
Malaise, fever, night sweats, arthralgias, anorexia, weight loss.
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INVESTIGATIONS
Haematology
ESR.
Mild anaemia.
Biochemistry
Elevated immunoglobulins.
Pathology
Histopathology diagnostic.
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MANAGEMENT
Medical
Some may just remit spontaneously.
Glucocorticoids (40-60mg prednisone) relieve symptoms
- no evidence that they increase survival.
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