TAKAYASU ARTERITIS - The Pulseless Disease

We all know how our Bollywood doctors check the 'Pulse' of a woman to confirm her pregnancy. Urinary beta-HCG levels are trivial markers of gestation for our white coat adorned, serious looking, bespectacled, on-screen doctors. But what if the lady has no pulse? How will our Doctor say, "We have good news for you"? They will now have to concentrate on a more serious condition - Takayasu's Arteritis.

What is Takayasu's Arteritis?

It is a progressive inflammation ( arteritis= artery + itis[inflammation] ) of the medium and large arteries of the body. It is named after the Japanese Ophthalmologist, Dr. Mikito Takayasu who first described it. The cause is unknown hence idiopathic ( with an idiotic pathology) but can be explained by an Autoimmune reaction ( antibodies which attack germs attack our own body ). In short it is a disease in which our body attacks our arteries.

What is it otherwise reffered to as?

It is also known as 'Aortic Arch Syndrome'; 'Non-specific Aortoarteritis' and 'Pulseless Disease'.

Why is it called pulseless disease?

The main supply of blood in our body is via the Aorta which is the largest artery originating from the left ventricle of the heart. It ascends upwards in the chest, arches and then descends to distribute blood in the abdomen. It gives off 3 main branches at the arch which are:

• Brachiocephalic Trunk - supplies right upper limb and right head & neck region.

• Left Common Carotid Artery - supplies left head & neck region.

• Left Subclavian Artery - supplies left upper limb. Pulse is felt mostly in the Radial artery (end branch of Subclavian Artery) in forearm and in the Carotid Arteries in the neck. In Takayasu's Arteritis, most commonly the Subclavian and Carotid Arteries which are branches of arch of Aorta are occluded hence it is called a 'Pulseless' disease.

Whom does it commonly affect?

It commonly affects Young Japanese Females of childbearing age and goes unnoticed for a long time, the mean age of onset being 30 yrs. In Japanese patients the Aortic Arch is more commonly affected, whereas the scenario in India shows more involvement of the Abdominal Aorta. Also, in Japanese patients, HLA-Bw52, an antigen shows genetic predisposition. It is often bilateral i.e. affecting both sides.

How does it present?

Most important symptoms include:

• Fever with joint pains (Similar to rheumatic fever).

• Absent pulses in the upper limb/limbs, neck which is associated with weakness in upper limbs.

• Increased Blood Pressure leading to Hypertension.

• Fainting with changes in position of neck and atrophy of face.

• Later it can cause seizures and hemiplegia/paralysis on one side. 

How is it differentiated from similar conditions?

Temporal arteritis: Affects arteries of head and usually in persons above 50 yrs.

Coarctation of Aorta: Birth defect involving narrow Aorta usually after Left Subclavian Artery branches out. Pulse delay is seen and imaging studies show the difference.

How is it identified and diagnosed?

MR angiography- Helps to visualize the vessel and see the obstruction.

Doppler study- Helps to check the flow of blood in the affected artery.

CT angiography- Helps in visualization.

What is the treatment?


Prednisolone- Reduces inflammation and supresses immune response.


Cyclophosphamide- Supresses immune response.


Vascular Reconstruction Surgery- Angioplasty(surgical replacement of diseased artery); Stenting; Aneurysm repair.


What complications can occur if timely treatment is not provided?


Stroke- Due to decreased blood supply to the brain.


Graft Stenosis or Occlusion- Recurrence of disease.


Organ failure- Due to ischemia/decreased blood supply.


Foetal injury- Due to decreased placental blood supply in pregnancy.


Complications of Hypertension.                                                                                                         

Valvular Heart Disease.


Conclusion


Takayasu's Arteritis has substantial morbidity and can be life threatening. Early detection through awareness and efficient diagnostic skills will help with early intervention and treatment of the disease.