Makindo Medical Notes

Weight loss, malaise, patient under 40, absent pulse and raised ESR = Takayasu's disease

About

• Takayasu arteritis is a chronic inflammatory disease of unknown origin, primarily affecting the aorta and its major branches. The disease is most common in young adults, with a median age of onset at 29 years.
• Women are eight times more likely to develop the condition than men, and it occurs more frequently in Asia and Africa compared to Europe and North America.

Pathophysiology the following stages:

• Active inflammation: Involves the aorta and its branches.
• Sclerotic stage: Characterized by intimal hyperplasia, medial degeneration, and obliterative changes.
• Most arterial lesions are stenotic, but aneurysms may also develop.

Affected Vessels

• The disease mainly involves the aorta and its branches, occasionally affecting the pulmonary artery.
• Inflammation most pronounced at the aortic arch, brachiocephalic vessels, and abdominal aorta.
• Diffuse or patchy inflammation, with affected areas separated by segments of normal aorta.

Clinical

• Fatigue, Headache
• Fever, Night sweats
• Arthralgia, Weight loss

Clinical signs of vascular insufficiency

• Claudication in the upper or lower extremities
• Absent pulses and diminished blood pressure in the arms - "pulseless disease"
• Bruits over affected arteries
• Hypertension, particularly from renal artery involvement
• Aortic valve insufficiency
• Coronary artery involvement causing angina or myocardial infarction
• Heart failure due to myocardial infarction, hypertension, or aortic insufficiency
• Carotid artery involvement leading to cerebral ischaemia or stroke
• Abdominal angina from compromised mesenteric circulation

Laboratory Findings

• Elevated C-reactive protein (CRP) levels
• Elevated erythrocyte sedimentation rate (ESR)
• Anaemia
• Mild leukocytosis
• Elevated immunoglobulin levels

Diagnostic Criteria

Diagnosis

The diagnosis is best confirmed using imaging techniques such as:

• Aortography
• CT angiography
• MR angiography

Treatment and Prognosis

• Corticosteroids (e.g., prednisone 60-100 mg/day) are the primary treatment during the acute inflammatory stage.
• If corticosteroids alone are ineffective, additional immunosuppressive medications like tocilizumab (162 mg/week) or methotrexate (15-25 mg/week) may be used.
• Percutaneous balloon angioplasty may help dilate short stenotic lesions, though restenosis is common.
• Surgery may be required to bypass or reconstruct vital arteries or treat aortic insufficiency, but it is best avoided during the inflammatory phase.
• Approximately 50% of patients experience a relapse within 10 years of diagnosis. The overall 15-year survival rate is about 85%, but drops to 65% for patients with major complications. Most deaths result from stroke, myocardial infarction, or heart failure.