Takayasu Arteritis


What is takayasu arteritis?

Takayasu arteritis is an uncommon disease occuring due to inflammation of the large blood vessel of the body which carries oxygenated blood from the heart. The wall of the aorta or its branches gets swelled up. Due to this, the blood is inadequately circulated in the whole body or the part of the body depending upon the affected blood vessels and resultant of this is the absence of pulse in hand, neck, leg or other part of the body.

takayasu arteritis image


Takayasu arteritis is also known as ‘pulselessness’. The incidence rate is high in Asian women. Numbers of male patient are less in comparison with female patients. It is often common in female at child bearing age, but it can also develop at the age between 10 to 20 years.

The reason behind the development of this disease is yet unknown. Researchers believe that due to immunological disorder, vasculitis is developed. Another possible cause is viral or bacterial infections.

Some paper also published wrong genetic sequence may also be a cause of takayasu arteritis. Further research is required for establishing the exact cause, but it is clear that takayasu arteritis leads to narrowing of the arteries which obstruct the blood flow.


Pathophysiology

In this disease, vasculities developed in aorta or its branches which followed a lesion development which is described as a panarteritis with intimal proliferation.

In advance of the lesion causes stenosis in the arterial fibers and leads to narrowing of the affected arteries. This causes obstruction in the blood flow. Depending upon the affected arteries the effect is coming up like renal artery stenosis causes hypertension.

The frequent findings with takayasu arteritis are cardiac disorder, including congestive heart failure, but cardiomyopathy, myocarditis, and pericarditis are also reported with takayasu arteritis.

In case of pulmonary diseases like the development of the pulmonary fibrosis, alveolar damage, pneumonia and interstitial, it has been reported that due to vasculities in the pulmonary artery present at right side mainly causes the onset of diseases.

Other possible diseases along with takayasu arteritis are hypotensive ischemic retinopathy, carotid stenosis, vertebrobasilar ischemia, microaneurysms and inflammatory bowel disease.

Rarely takayasu arteritis causes glomerulonephritis, polymyositis, systemic lupus, rheumatoid arthritis, polymyalgia rheumatic, ankylosing spondylitis and Still disease.

Symptoms of takayasu arteritis

Usually, the symptoms of takayasu arteritis is divided into two stages

In stage 1, the included symptoms are:

  • Tiredness
  • Wight loss without finding of any simple reasons
  • Muscle spasm
  • Pain in the joints
  • Slightly increased body temperature

Some patients are not developing stage 1 symptoms and drastically stage 2 symptoms appear directly, following are the stage 2 symptoms:

The symptoms of stage 2 are all mainly developed due to narrowing of the arteries. For this reason the oxygenated blood circulation is less in the organs and tissues and following symptoms arise:

  • During any type of physical activity, the upper or lowers limbs become easily fatigue and pain symptoms started.
  • Vertigo
  • Sudden unconsciousness
  • Headache
  • Memory impairment
  • Concentration problem
  • Breathing shortness
  • Visual disturbances
  • Hypertension
  • Blood pressure difference between two limbs
  • Difficult to get pulse, especially in the wrist.
  • Decrease red blood cells counts
  • Pain in the chest
  • Abdominal spasm

Types/Classification

Depending upon the angiographic result, takayasu arteritis is classified into six categories,


  • Type -1 – Inflammation developed in the branches of the aortic arch.
  • Type 2a – Inflammation developed in the ascending aorta, aortic arch and its branches
  • Type 2b – Inflammation developed thoracic descending aorta including blood vessels involved in 2a region.
  • Type 3– Inflammation developed in renal arteries, abdominal aorta, thoracic descending aorta or a combination of both
  • Type 4 – Inflammation developed in renal arteries, abdominal arteries or in both arteries
  • Type 5 – Inflammation occurs at the whole aorta and its branches.

takayasu arteritis

Treatment

Before start the treatment, the following diagnosis is need to conduct including

  • Monitoring of blood pressure
  • Blood test to diagnose the anaemic condition
  • Chest X-ray, which usually provides an image of abnormal throbbing found in the affected area.
  • Angiogram is conducted for diagnose the affected blood vessel.

The aim of the treatment of takayasu arteritis is to prevention of further narrowing of the arteries by controlling the vasculities.

Different steroidal drugs, including glucorticoids such as prednisolone, prednisones etc are prescribed for controlling the inflammation. Steroidal drugs are having different side effects, so depending upon the severity of the disease condition; doctors decide the dose and duration of the medications.

As the steroidal drugs are much more long term side effects, to lessen the effect doctors also prescribed immune-suppressive medications like Methotrexate, Cyclophosphamide, Azathioprime etc. For some cases, low dose of blood thinning agents such as aspirin also use to treat takayasu arteritis.

Depending upon the patients diagnostic reports, antihypertensive drugs, anti-cholesterol drugs also prescribed.

In case of total blockage of blood flow need surgical intervention like angioplasty or bypass surgery. In angioplasty, stent may or may not place for widening the affected arteries. In bypass surgery, the direction of the blood flow is changed from blockage arteries.

Prognosis

Complete cure of takayasu arteritis is not possible, but right diagnosis and early initiation of treatment can prevent the further complication and provide quality of life to the patient. The mortality rate is less in adult in comparison to children.


References

  1. Who gets Takayasu’s Arteritis (the “typical” patients)?, Classic symptoms of Takayasu’s Arteritis, What causes Takayasu’s Arteritis?, How is Takayasu’s Arteritis diagnosed?, Treatment and Course of Takayasu’s Arteritis, What’s new in Takayasu’s Arteritis? at http://www.hopkinsvasculitis.org/types-vasculitis/takayasus-arteritis/ by David Hellmann, M.D.
  2. http://www.medicinenet.com/takayasu_disease/article.htm
  3. http://www.mayoclinic.org/diseases-conditions/takayasus-arteritis/basics/symptoms/con-20028085
  4. Takayasu’s Arteritis -> Overview, Presentation, DDx, Workup, Treatment, Medication at http://emedicine.medscape.com/article/332378-overview#a4 by Jefferson R Roberts, MD; Chief Editor: Herbert S Diamond
  5. Paul A. Monach, MD, PhD, and Peter A. Merkel, MD, MPH, and reviewed by the American College of Rheumatology Communications – See more at: http://www.rheumatology.org/I-Am-A/Patient-Caregiver/Diseases-Conditions/Takayasus-Arteritis#sthash.mcMDmKL9.dpuf

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