OP16. TAKAYASU ARTERITIS CLINICAL SPECTRUM: NORTH AMERICAN EXPERIENCE

• Published in: Rheumatology (Oxford, England) Vol. 44; no. suppl-3; p. iii7
• Main Authors: Soto, M. E., Flores-Suárez, L. F., Reyes, P. A.
• Format: Journal Article
• Language: English
• Published: Oxford Oxford University Press 01.07.2005; Oxford Publishing Limited (England)
• ISSN: 1462-0324; 1462-0332
• DOI: 10.1093/rheumatology/keh741

Objective: To provide clinical data of a cohort of 125 patients with Takayasu arteritis (TA) seen from 1972–2005. Methods: Retrospective chart review of 108 women and 17 men with TA. All data concerning clinical presentation and evolution were recorded and descriptively analysed. Results: Female:male ratio was 6.3:1, 93% of patients were ≤40 years old. In 119 panaortogram was performed and 5 were evaluated with magnetic angioresonance. Four ACR criteria were met in 23, 5 in 60 and 42 had all six criteria. Anatomic lesions were found in the following arteries: right subclavian (38%), left subclavian (65%), right carotid (28%), left carotid (42%), any vertebral (21%), pulmonary arteries (12%), coronaries (13%), renal (47%), iliac (23%), abdominal aorta (34%), superior mesenteric (18%), inferior mesenteric (11%). Numano's type V disease was the most common (66%) followed by type I (21%). Most common symptoms and signs grouped by systems were: general (malaise, fever, sweating, weight loss) (35%); neurologic: headache (70%), dizziness (56%), syncope (35%), seizures (20%), stroke (1%); ocular: amaurosis (23%), cataracts (10%); cardiopulmonary: systemic hypertension (54%), dyspnea (53%), precordial pain (31%), limb claudication (34%), angiodinia (22%); cutaneous: erythema nodosum (18%). Laboratory features: high ESR (55%), normocytic normochromic anaemia (46%), elevated C-reactive protein (29%), leukocytosis (21%). Activity was recorded in 28 patients (22%) at some time during their disease based on a proposed clinical score system that includes clinical and laboratory parameters. Ischaemic cardiopathy was seen in 17 patients, 15 of them had acute myocardial infarction. Valvular disease was evaluated in 86 patients with aortic regurgitation in 37%, mitral insufficiency-22%, tricuspid regurgitation-15%. Those that required therapy (n = 56) received prednisone (25%), prednisone + methotrexate (15%), prednisone + cyclophosphamide (4%), prednisone + azathioprine (1%). None has been treated with biologicals. Mortality was high (25%), causes being: myocardial infarction-5, non-vascular postsurgical complications-5, chronic renal failure-3, complications during revascularization-3, cerebrovascular disease and acute pulmonary oedema-2 each, subaracnoidal haemorrhage, hyperperfusion syndrome, aortic dissection, pulmonary thromboembolism, intestinal ischaemia and septic shock-1 each, unknown-5. 10% have been lost to follow-up. There have been 4 vascular surgeries, 2 succeeding in reperfusion of compromised territories. Four coronary bypass surgeries were done and 1 coronary stent placed, all unsuccessful. Vascular stenting has been done in 8, all had poor results. Based on analysis of 110 cases, incidence from 1976 to date is 0.9/100,000/year, survival at 5 and 10 years is 85% and 81%, respectively. Life expectancy is reduced when aortic regurgitation and coronary involvement are present. Conclusions: Extensive disease is common in our series with arterial renal stenosis in almost half the cases which might influence the development of difficult-to-treat arterial hypertension. We need to detect hypertension and valve disease earlier which confer a bad prognosis as does Numano type V disease. High mortality is seen in our population due to chronic course of disease and few medical or surgical options at that stage. In active patients, remission was achieved although therapy is still unsatisfactory to avoid the development of fibrosis.