Name
TAKAYASU ARTERITIS
DESCRIPTION
DETAIL
OTHER TESTS : * ESR - RAISED * IMMUNOGLOBULIN LEVELS - RAISED * BLOOD TESTS - MILD ANEMIA * BIOPSY OF INVOLVED VESSELS - DIAGNOSTIC * ARTERIOGRAPHY
TYPENOTES
CAN CAUSE ANGINA DUE TO MICROVASCULAR ABNORMALITIES.Medical Care: Therapeutic intervention includes corticosteroids with or without cytotoxic agents. " Corticosteroids are the mainstay of therapy for active Takayasu arteritis, and some patients may require additional cytotoxic agents to achieve remission and discontinue steroid treatment. o Corticosteroids are started at 1 mg/kg/d PO or divided bid/qid and tapered over weeks to months as symptoms subside. Long-term low-dose corticosteroid therapy may be required. o Osteoporosis prevention when patients are started on corticosteroids should be seriously considered. " Cytotoxic agents are used for patients with steroid resistance or relapsing Takayasu arteritis. These agents are usually continued for one year after remission and are then tapered to discontinuation. Agents and doses are as follows: o Azathioprine 1-2 mg/kg/d PO o Methotrexate 7.5-25 mg/wk PO or IM o Cyclophosphamide 2 mg/kg/d PO (should be reserved for patients with the most severe and refractory disease states) " Cyclosporin A also has been used in steroid-resistant patients at initial doses of 5 mg/kg/d and then 2-3 mg/kg/d for maintenance. " Mycophenolate mofetil (2 g/d PO) has been used in patients with Takayasu arteritis resistant to steroids and other immunosuppressant drugs. " In an uncontrolled series of 15 patients, adjunctive treatment with anti-tumor necrosis factor (TNF) agents was effective in patients with active, relapsing Takayasu arteritis despite treatment with steroids and multiple other immunosuppressive agents. The initial dose of etanercept was 25 mg twice weekly (7 patients), and infliximab (11 patients [3 were switched from etanercept to infliximab]) was given at 3 mg/kg initially and at 2 weeks, 6 weeks, and every 8 weeks thereafter. In 9 of the 14 responders, an increase in the anti-TNF dosage was required to sustain remission. These preliminary results suggest that anti-TNF therapy may be a useful adjunct to corticosteroids in the treatment of patients with Takayasu arteritis and require further studies. A larger randomized controlled study of anti-TNF therapy for Takayasu arteritis is planned. " Strict management of traditional cardiovascular risk factors such as dyslipidemia, hypertension, and lifestyle factors is mandatory to minimize secondary cardiovascular complications, which are the major cause of death in this disease. Additionally, low-dose aspirin may have a therapeutic effect in large vessel vasculitis. Surgical Care: " Critical stenotic lesions should be treated by angioplasty or surgical revascularization during periods of remission. " Indications for surgical repair or angioplasty are as follows: o Renovascular stenosis causing hypertension o Coronary artery stenosis leading to myocardial ischemia o Extremity claudication induced by routine activity o Cerebral ischemia and/or critical stenosis of 3 or more cerebral vessels o Aortic regurgitation o Thoracic or abdominal aneurysms larger than 5 cm in diameter o Severe coarctation of the aorta " Percutaneous transluminal coronary angioplasty is followed by restenosis at the angioplasty site within 1-2 years in a substantial number of patients. " Bypass graft procedures have the best long-term patency rate.
RELATED DISEASE
Not Available Disease
DISEASE
INVESTIGATION
PERIPHERAL VESSELS DOPPLER, COMPLETE BLOOD COUNT, BIOPSY