This book describes Pulseless Disease, Diagnosis and Treatment and Related Diseases
Pulseless Disease (Takayasu arteritis) is an unusual inflammation of large arteries such as the aorta and its major branches.
The aorta is the artery that transports blood from the heart to the rest of the body.
Pulseless Disease (Takayasu arteritis) mostly involves the aorta and the pulmonary artery (which sends blood to the lungs).
The major arteries or branches that arise from the aorta may also be involved:
1. The subclavian arteries that supply the arms,
2. Renal arteries to the kidneys,
3. Coronary arteries in the heart and
4. Carotid arteries in the head and brain.
The widening of the aorta leads to failure of the aortic valve in the heart, needing replacement in some patients.
In 90% of patients one or more of these arteries become narrowed or blocked, hence Takayasu arteritis (named after is also called "Pulseless disease".
In 25 per cent of patients part of an artery may bulge out, forming an aneurysm.
The cause of Pulseless Disease (Takayasu arteritis), and why a patient forms the disease at any one time stays not known.
Pulseless Disease (Takayasu arteritis) is not a genetically inherited disorder but most likelyan autoimmune disorder.
This indicates the body's immune system incorrectly attacks the healthy tissue.
The inflammation affects white blood cells attacking the wall of the artery causing it to damage and scarring.
Symptoms
The early symptoms of Pulseless Disease are normally non-specific and may be one or more of:
1. Malaise
2. Profound fatigue,
3. Fever,
Additional symptoms can be:
1. Dizziness
2. Shortness of breath,
3. Cramping pain in the arms, legs or chest on exertion.
4. Arm weakness or pain with use
5. Chest pain
6. Vision changes
7. Decreased radial pulses
Signs
The main sign is a systolic blood pressure (SBP) difference >10 mm Hg between the arms.
1. Peripheral pulses may not be felt.
2. Arterial bruits over any large artery and bruit of aortic regurgitation.
3. High BP in 50%
Diagnosis:
For diagnosis the patient should fulfill 3 or more of the criteria listed:
1. Onset of disease ≤ 40 years
2. Claudication of an extremity - caused by narrowed blood vessels
3. Reduced brachial artery pulsation
4. Difference in systolic blood pressure >10 mmHg between the arms
5. Aortic or sub-clavian artery bruit
6. Angiographic abnormality
The medical examination most often detects reduced or absent pulses in the arms and less often in the legs.
Using a stethoscope, bruits may be heard, over the neck, chest or kidneys indicating narrowed arteries
The raised erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP) indicate inflammation.
Angiography is the main diagnostic test.
It appraises the arterial lumen only
The obligatory criterion is angiography (conventional, CT or MRI) of the aorta or its main branches and pulmonary arteries indicating aneurysm or dilatation, narrowing, occlusion or thickened arterial wall
Treatment:
The treatment is directed at improving symptoms and avoiding scarring to the blood vessels.
When there is active arterial inflammation, the treatment is first treated with high doses of steroids.
Immunosuppressive drugs (azathioprine, mycophenolate, methotrexate or leflunomide) are often added.
For refractory disease, biological therapies, such as tumor necrosis factor inhibitors (etanercept and infliximab) have been used effectively
Medicines to lower blood pressure are often...