Occlusive thromboaortopathy (Takayasu disease): clinical and angiographic features and a brief review of literature.

Abstract

The aim of this study was to evaluate the diagnostic criteria and angiographic classifications of Takayasu arteritis by presenting the clinical, angiographic, and prognostic findings and a prospective follow-up of 78 patients. Occlusive thromboaortopathy or Takayasu arteritis is a large vessel vasculitis. The disease is systemic with an autoimmune and genetic etiology. The complete clinical and angiographic manifestations are reported for 78 cases based on diagnostic criteria of the American College of Rheumatology with a mean 6 +/- 3.2 years follow-up. The mean age was 34.7 and female:male ratio was 3.6:1. According to National Institute of Health criteria, 61.5% of patients were in the acute phase of disease with systemic symptoms such as fever, weight loss, malaise, and elevated C-reactive protein levels. Immunologic markers, such as antinuclear antibody and antineutrophil cytoplasmic antibodies, were negative. The tuberculin test result was positive in 47%. Vascular bruit was present in 89%. Almost all patients had stenoses, occlusions, or aneurysmatic changes of the aorta and its main branches. Hypertension was detected in 58% and left ventricular hypertrophy was initially present in 22 (28%) patients. The angiographic manifestations were classified as type I, cervicobrachial type with 20 cases (25.6%); type II, thoracoabdominal type with 13 cases (16.6%); type III, peripheral type with 10 cases (12.8%); and type IV, generalized type with 35 cases (44.8%). The coronary arteries were involved in 6 cases, pulmonary arteries in 11 initially 5 in follow-up (16 cases), and renal arteries in 28 cases, respectively. A good correlation of the clinical manifestations and the prognosis was observed. During follow-up, five patients suffered from myocardial infarction, six had cerebrovascular accident, seven patients underwent aortic valve replacement, and six patients died (mortality rate, 7.6%). The specificity and sensitivity of diagnostic criteria were 94% and 76%, respectively. In contrast to ours and Nasu's classification in the new classification of Numano, some angiographic types and subtypes of Takayasu arteritis are not present in our patients.