History -27y/F with generalized weakness
Findings - Angiography showing narrowing of aorta and its major branches

Diagnosis - Aortoarteritis

Also known as Takayasu’s disease/ Pulseless disease

It is a primary arteritis of undetermined etiology, most commonly affecting the aorta, its major branches and the pulmonary artery. This usually  leads to luminal stenosis ,occlusion ,dilatation and aneurysm formation of involved vessels It’s the only form of aortitis that produces stenosis /occlusion of aorta.

The diagnosis depends on the typical angiographic morphology, history or presence of constitutional symptoms suggestive of a systemic illness.

Ishikawa’s criteria for the clinical diagnosis of Takayasu’s disease

Age <40 year : Age < 40 year at diagnosis or at onset of "characteristic signs and symptoms" of one month duration in patient history

1.Left mid subclavian artery  :  The most severe stenosis or occlusion present in the mid portion from the point one cm proximal to the left vertebral artery orifice to that three cm distal to the orifice determined by angiography. 

2. Right mid subclavian artery lesion :  The most severe stenosis or occlusion present in the mid portion from the right verterbral artery orifice to the point 3 cm distal to the orifice determined by angiography. 

1. High ESR  :   Unexplained persistent high ESR>20 mm/h (Westergren) at diagnosis or presence of evidence in patient history.

2. Carotid artery tenderness  :  Unilateral or bilateral tenderness of common carotid arteries by physician palpation; neck muscle tenderness is unacceptable.

3. Hypertension :  Persistent blood pressure 140/90mmHg brachial or > 160/90mmHg popliteal at age <40 year. Or presence of the history at age <40 year. 

4. Aortic regurgitation or annuloaortic ectasia : By auscultation or Doppler echocardiography or angiography   .  

5. Pulmonary artery lesions : By angiography or two dimensional echocardiography. Lobar or segmental arterial occlusion or equivalent determined by angiography or perfusion scintigraphy; or presence of stenosis, aneurysm, luminal irregularity or any combination in pulmonary trunk or in unilateral or bilateral pulmonary arteries determined by angiography.

6. Left mid common carotid lesion : Presence of the most severe stenosis or occlusion in the mid portion of 5cm in length from the point 2 cm distal to its orifice determined by angiography.

7. Distal brachiocephalic trunk lesion: Presence of the most severe stenosis or occlusion in the distal third lesion determined by angiography.

8 . Descending thoracic aorta lesion :  Narrowing, dilatation or aneurysm, luminal irregularity or any lesion combination determined by angiography; tortuosity alone is unacceptable.

9. Abdominal aorta lesion :  Narrowing, dilation or aneurysm, luminal irregularity or any combination and absence of lesion in aorto-iliac region consisting of 2cm of terminal aorta and bilateral common iliac arteries determined by angiography; tortuosity alone is unacceptable.    

The proposed criteria consist of one obligatory criterion, two major criteria and nine minor criteria. In addition to the obligatory criterion, the presence of two major criteria, or one major and two or more minor criteria or four more minor criteria suggests a high probability of the presence of Takayasu’s disease.

 

Treatment : Steroids

                      Angioplasty only after decline of active inflammation.