What is Takayasu’s Disease
Takayasu’s disease is named in honor of Japanese ophthalmologist Mikito Takayasu, who first reported a case of the disease in 1905. His patient was a 21-year-old woman with retinal vessel changes and decreased pulses in branches of the aortic arch.
Definition
It is a chronic inflammatory, granulomatous panarteritis of unknown cause involving the elastic arteries.
It commonly involves aorta and its major branches, carotid, ulnar, brachial, radial and axillary. Occasionally, may involve pulmonary artery, rarely abdominal aorta, renal artery resulting in obstruction. Common in female at the age of 25–30 years. Mainly found in asians.
Clinical Features
• General- fever, malaise, weight loss, arthralgia, myalgia, dizziness, giddiness, headache, syncope, seizure, claudication in the upper limb.
• There may be aortic regurgitation, renal artery stenosis or anginal pain. Hypertension is found in 32–93%. There may be pericarditis, myocardial infarction, congestive cardiac failure, etc.
Ophthalmologic findings—Ophthalmologic examination may show retinal ischaemia, retinal hemorrhages, cotton-wool exudates, venous dilatation and beading, microaneurysms of peripheral retina, optic atrophy, vitreous hemorrhage, and classic, wreath like peripapillary arteriovenous anastomoses.
Signs
• Pulse—All peripheral pulses are absent or feeble in the upper limb. In the lower limbs, pulse
is present.
• BP—Not recordable in upper limbs.
• Bruit—may be present over both carotids.
• Locomotor system—small joints of hands, wrists and elbows are mildly tender.
Investigations
• CBC (high ESR and normocytic normochromic anaemia).
• Chest X-ray shows cardiomegaly and widening of aorta.
• Aortography of aortic arch and its branches, renal angiogram shows narrowing, coarctation
and aneurismal dilatation.
• Serum immunoglobulin is high.
Treatment
• High-dose prednisolone—40–60 mg daily or 1–2 mg/kg.
• If refractory to steroid or difficult to taper steroid, methotrexate up to 25 mg weekly.
• Reconstructive vascular surgery in selected case.
Prognosis
With appropriate treatment, the 5 year survival is 83%.