Heart and vessels. Supplement最新文献

The guidelines for medical treatment of Takayasu arteritis established in 1987 by the Systemic Vascular Disorders Research Committee, Ministry of Health and Welfare of Japan are presented. The first part of the guidelines concerns treatment with adrenocorticosteroids and the second part concerns other medical treatment. A review of the literature referring to steroid therapy and other medical treatment of Takayasu arteritis is also included.

This communication reviews the clinical and pathological features of coronary artery lesions in Takayasu arteritis. The incidence of coronary artery involvement has been reported to be 9% to 10%, and is observed mainly in autopsy cases because coronary artery disease is usually not evident until the occurrence of angina pectoris or myocardial infarction, or after the onset of congestive heart failure. On the basis of pathological features, the following three types of coronary artery lesions can be distinguished: type 1, stenosis or occlusion of the coronary ostia and the proximal segments of the coronary arteries; type 2, diffuse or focal coronary arteritis, which may extend diffusely to all epicardial branches or may involve focal segments, so-called skip lesions; and type 3, coronary aneurysm. Most of the coronary artery lesions in Takayasu arteritis are of type 1. Narrowing of the coronary arteries is mainly due to the extension of the inflammatory processes of proliferation of the intima and contraction of the fibrotic media and adventitia from the ascending aorta. In some cases, coronary stenosis may be caused by coronary arteritis as skip lesions in Takayasu arteritis, but even in these cases the lesions have been reported to affect mainly the proximal segments of the coronary arteries. Diffuse lesions of the coronary artery and coronary artery aneurysm seem to be very rare in Takayasu arteritis. Other causes of coronary ostial stenosis, coronary arteritis and coronary artery aneurysm are also discussed.

A total of 530 patients with Takayasu arteritis were studied. Among 346 patients who were diagnosed by aortography, the female to male ratio was 2.9 to 1, and the age of onset ranged from 5 to 45 years. Three hundred and eighteen (60%) patients with secondary hypertension, including renovascular hypertension in 281, and 197 (37.2%) with pulseless extremities were found in the series. All the patients were treated with medical or surgical procedures. Surgical treatment is preferred if clinical status of the patient permits. The patients were followed for 1-29 years (average 7.8 years). There were 55 deaths (10.4%) in this series. Cerebral hemorrhage was found as a common cause of death. Five-year and ten-year survival rates were 93.1% and 91.1%, respectively.

Little attention has been paid to the pathological features of the pulmonary artery in Takayasu arteritis. Autopsy specimens of 6 cases of this disease were studied. Lesions were found in the aortic arch and its brachiocephalic branches in all cases and in both the aortic arch and thoracoabdominal aorta in 5 cases. The pathohistologic characters of the pulmonary artery were very similar to those of the systemic artery. Stenosis-recanalization, so-called blood vessels-in-blood vessels, of the pulmonary elastic arteries were found in four cases. These lesions were not observed in the systemic arteries, and most of the newly formed channels in them seemed to be branches of bronchial arteries. Luminal obstruction of pulmonary muscular arteries was observed in 4 cases, cellular arteritis of muscular arteries in 2 cases, and angiomatoid dilatation of small blood vessels in 2 cases. Thus in this study we found peculiar stenosis-recanalization lesions of the pulmonary elastic arteries, and also showed that the pulmonary elastic and muscular arteries are frequently involved in Takayasu arteritis. These findings suggest that pulmonary hypertension could influence morbidity and long-term mortality in this disease.

Takayasu arteritis is a common cause of renovascular hypertension in India. Sensitization to infective agents, particularly mycobacterium tuberculosis or autoimmune disturbances have been incriminated in its pathogenesis. Twenty patients of Takayasu arteritis along with groups of normal individuals, patients of essential hypertension, autoimmune disorders, tuberculosis, and healthy tuberculin reactors were studied. Besides detailed immunological profiles including LE cell phenomenon, serum complement C3 levels, antibodies to single (SS) and double stranded (DS) DNA, rheumatoid factor, lymphocyte subsets, blast transformation to antigens including, phytohemagglutinin, PPD, pokeweed, and purified human aortal antigen (PHAA) were examined. Soluble protein from human aorta was fractionated into 9 peaks by DEASE-52 and Sephadex G-75 chromatography, and 25 micrograms of major protein fraction-II was used for blast transformation study. Blast transformation by PHAA was higher in Takayasu arteritis as compared to all other groups (P < 0.05). Blast transformation to PPD showed wide variation in all the groups, and was significantly higher only in tuberculin reactors as compared to controls. These observations support aortal sensitization to PHAA playing a role in pathogenesis of Takayasu arteritis and do not relate tuberculosis to Takayasu arteritis, at least immunologically. In addition, the ratio of CD-4 positive to CD-8 positive lymphocytes changing in favor of the former and the concomitant increase in B lymphocytes favor the presence of autoimmune disturbances in Takayasu arteritis.

It is important in patient management to evaluate coronary arterial involvement in aortitis syndrome. Twenty-one cases of aortitis syndrome who experienced chest pain were examined using exercise thallium scintigraphy. The patients were divided into 4 groups according to the angiographic findings. There were five patients with left main coronary arterial involvement (group A), four with left or right coronary arterial involvement (group B), nine with aortic regurgitation (group C), and three with pulmonary arterial involvement (group D). In groups A and B, all patients had positive ECGs and thallium perfusion defects. Group A patients showed extensive anterolateral perfusion defects, which were compatible with left main coronary arterial involvement. Groups C and D patients, who had normal coronary arteries, showed no remarkable perfusion defects although five had positive ECG findings. Thus, the sensitivity and specificity of exercise scintigraphy for detection of myocardial ischemia were 9/9 and 12/12, while those of stress ECG were 9/9 and 7/12 (58%), respectively. it is recommended that exercise thallium scintigraphy be used for detecting clinically occult but significant coronary arterial involvement in aortitis syndrome with chest pain.

Seventy cases of Takayasu arteritis in Korean children are reported. There were 57 females and 13 males (male-to-female ratio; 1:4.4). The youngest patient was a 3-year-old female. Family history was positive in one patient. The most common chief complaints on admission were dyspnea, headache, palpitation, and edema which were due to hypertension and congestive heart failure. Hypertension was seen in 65 out of 70 patients (92.8%). The abdominal aorta, thoracic aorta, and renal arteries were the most commonly involved sites in these children. Two patients had nephrotic syndrome. The frequency of positive tuberculin reaction was much higher in children with Takayasu arteritis compared with the general population, and the intensity of the reaction was also stronger. The majority of the patients required immediate medical treatment to control congestive heart failure due to hypertension at initial presentation. When ESR was elevated, corticosteroid was administered. Surgical treatment showed good results in six out of ten cases. Percutaneous intraluminal angioplasty was effective for lowering the blood pressure in six out of nine cases. In three cases, restenosis occurred and angioplasty was repeated in two cases.

Clinical and angiographic features of Takayasu arteritis were investigated in 129 Korean patients. This disease affects females more frequently than males, in a ratio of 6.6 to 1. Of the total number of patients, 51 were in the third decade, 27 in the fourth decade, and 23 in the second decade. Common clinical symptoms were headache (60%), exertional dyspnea (42%), dizziness (36%), and malaise or weakness (34%). Takayasu arteritis affected the abdominal aorta (46%) and descending thoracic aorta (37%) more frequently than the ascending aorta (1%) and aortic arch (2%) According to Ueno's classification based on aortographic findings, the 129 patients were divided into type I (37), type II (25), and type III (67). Among the 48 patients who had coronary angiography, 11 (23%) showed coronary arterial involvement. Because the clinical features are determined by the extent and severity of the specific artery involved in the occlusive phase of the disease, total aortography including coronary angiography is very important in the initial evaluation of Takayasu arteritis.