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Takayasu's disease manifestations , investigations and treatment

This article is to discuss Takayasu's disease regarding its incidence , clinical picture , recommended investigations , methods of treatment and prognosis .
• ·It is called pulseless disease or aortic arch syndrome .
• It is a chronic granulomatous panarteritis affecting the aorta and its branches and the carotid, ulnar, brachial and radial arteries.
• It occurs in young females.
 It is rare except in Japan.

How to diagnose Takayasu's disease ? what are the symptoms and signs?

Clinical Picture 


(female: male ratio 8 : 1), age (25 - 30 yrs)
• Fever, weight loss, anaemia, and arthralgia.

• Angina, arm claudication.
• Hypertension - Aortic incompetence.
• Absent peripheral pulses.

Investingations

1- Hb  : low due to chronic disease , TLC : high  , ESR : high.
2- Angiography ---> narrowing of the vessels (four types):
• Type I in aortic arch.              • Type II in descending aorta .
• Type III mixed (I + II).           • Type IV involves the pulmonary artery.

Treatment: 

• Steroid and cyclophosphamide .
• Reconstructive vascular surgery should be avoided during periods of active inflammation.

Prognosis: Good in more than 90% of cases.
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Dr.Tamer Mobarak

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