Heart and vessels. Supplement最新文献

To investigate genetic factors involved in the pathogenesis of Takayasu arteritis, patients in the Japanese population were examined for HLA-A, -B, and -C alleles by serological typing and for HLA-DR, DQ, and DP alleles by DNA typing using polymerase chain reaction (PCR)/sequence-specific oligonucleotide probe (SSOP) analysis. The frequencies of HLA-Bw52, DRB1*1502, DRB5*0102, DQA1*0103, DQB1*0601, and DPB1*0901 alleles were significantly increased and the frequencies of HLA-Bw54, DRB1*0405, DRB4*0101, DQA1*0301, and DQB1*0401 alleles were significantly decreased. Strong linkage disequilibria among the increased alleles and among the decreased alleles were evident in the Japanese population. Therefore, the haplotype of HLA-B252-DRB1*1502-DRB5*0102-DQA1*0103-DQB1++ +*0601-DPA1*02-DPB1*0901 may confer susceptibility to Takayasu arteritis while another haplotype of HLA-Bw54-DRB1*0405-DRB4*0101-DQA1*0301-DQB1++ +*0401 may confer resistance to the disease. These observations clearly indicate that HLA-linked gene(s) are involved in the development of Takayasu arteritis.

The role of surgical therapy for Takayasu arteritis remains controversial. From 1973-1991, 23 patients with Takayasu arteritis have been treated at the University of Southern California. Twelve patients have required 17 arterial reconstructions for symptomatic complications of arterial disease refractory to medical therapy. Indications for operation have included renovascular hypertension (7), extremity ischemia (5), cerebrovascular insufficiency (2), dilated ascended aorta with aortic insufficiency (1), thoracic aortic aneurysm (1), and abdominal aortic aneurysm (1). Long-term clinical follow-up has demonstrated uniform symptomatic improvement. Fifteen of seventeen arterial reconstructions are still patent. Surgical treatment of symptomatic Takayasu arteritis is highly effective. Excellent long-term graft patency can be expected following arterial reconstruction.

Percutaneous transluminal balloon angioplasty (PTBA) was performed in 87 patients for 111 stenotic lesions due to Takayasu arteritis. Of the lesions attempted for dilatation, 35 were in the aorta, 64 in renal arteries, 9 in subclavian, and 3 in common iliac arteries. The stenosis of aorta could be successfully dilated in 33 of 35 (94.3%) patients with fall in peak systolic pressure gradient (PSG) from 77.7 +/- 28.4 mmHg to 26.4 +/- 20.6 mmHg (P < 0.001) and increase in luminal diameter from 4.7 +/- 2.4 mm to 10.1 +/- 4.1 mm (P < 0.001). On hemodynamic and angiographic restudy in 20 patients at 3-24 months (mean 7.7 +/- 4.1 months) further fall in PSG (> or = 15 mmHg) was observed in 7 patients, no significant change in 12 patients and restenosis with increase in PSG in one patient which could be successfully redilated. Late restudy at 36-60 months (mean 43 +/- 9.4) in six patients showed continued relief of stenosis (mean PSG 8.8 +/- 7.8 mmHg). Of the 64 stenotic lesions of the renal arteries, 58 (90.6%) could be successfully dilated with decrease in stenosis from 89.1 +/- 10.1% to 29.9 +/- 14.9% (P < 0.001). Follow-up intra-arterial digital subtraction angiography in 25 patients at a mean follow-up period of 13.1 months (range 3-29 months) showed restenosis in 5/36 (13.9%) lesions which could be successfully redilated. Angioplasty was also successful in dilating 8/9 (88.9%) subclavian and all 3 common iliac artery stenosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Takayasu arteritis is the commonest cause of renovascular hypertension in India. The clinical and radiological features, complications and course of 83 patients (51 females, 32 males) seen during the period from 1972-1990 are described in this study. The age of the patients ranged from 5 to 53 years with the mean +/- SD of 26.9 +/- 9.7. Hypertension (n = 50) and the related symptom of headache (n = 40), dyspnea (n = 24), and giddiness (n = 20) were common at presentation. Twelve patients were in congestive cardiac failure. The symptoms of activity with fever and arthralgia were present in only 16% contrary to reports from Japan and Mexico. Abnormal arterial pulses and bruit over abdominal (37%) or extra abdominal great arteries (25%) were useful clinical clues to suspect Takayasu arteritis. Rapid sequence intravenous urography was a sensitive screening procedure and predicted correctly the presence of renovascular disease in 80% of the patients. The diagnosis was confirmed on aortography in 72. In the rest, the clinical features and autopsy findings confirmed the same. The four patterns of the disease based on the anatomical extent of involvement were recognised. These were: type I (n = 8) with involvement of aortic arch and its branches, type II (n = 25) descending thoracic and abdominal aorta type III (n = 46) combination of I and II and type IV (n = 4) pulmonary artery in addition to any of the above.(ABSTRACT TRUNCATED AT 250 WORDS)

Takayasu arteritis occurs with a strong predilection for women and particular geographic areas, and as related to the etiology of the disease, association of HLA antigens has been suggested. In the present study, the authors investigated the association of Takayasu arteritis with class I and class II HLA antigens in 59 Korean patients with this disease. Increased frequencies of HLA-Bw52 (chi 2 6.213, P < 0.02), Cw6 (chi 2 4.132, P < 0.05), DR7 (chi 2 4.506, P < 0.04), and DQw2 (chi 2 7.327, P < 0.01) were observed in the patient group as compared to the control group of healthy Koreans. In the Korean population, 2 risk factors in the HLA system for developing this disease appear to be (1) Bw52 and (2) DR7 and a probable haplotype of Cw6, B13, DR7, DQw2. Previous studies of the Japanese population revealed association of Bw52 and class II HLA antigens (DR2, Dw12), which are in linkage disequilibrium with Bw52. It is of interest that in the Korean population, class II antigens (DR7, DQw2), which are not linked to BW52, are associated with the disease. This finding suggests that the disease susceptibility gene of Takayasu arteritis is located between the HLA-B locus and HLA-DR, DQ loci.

A Japan-Korea cooperative survey on Takayasu arteritis has shown some differences in the features between Japanese and Korean patients with this disease. In angiographic findings, Japanese patients more frequently had lesions at the aortic arch and/or its branches (58% of 75 cases), while, in Korean patients, the abdominal aorta is the site of relatively frequent lesions (30% of 112 cases). Higher occurrence of HLA-Bw52 was found in Japanese patients in comparison with Korean patients (46% vs 15%). The presence of HLA-Bw52, however, might have a close association with Takayasu arteritis in Korea as well as in Japan. The complications in 126 Japanese and 88 Korean patients were also compared. The complications occurring with higher frequency in Japanese patients were aortic regurgitation, ischemic heart disease, and visual disturbances, while, in Korean patients, the more frequent complications were renovascular hypertension as well as hypertension of some other etiology.

Takayasu arteritis is a primary inflammatory disease of elastic arteries such as the aorta, its larger branches and the pulmonary artery trunk. According to our recent statistical survey of autopsy cases in Japan, the frequency of the disease in all autopsy cases was approximately 0.033% and the sex ratio was 1:4.5. The most frequent ages of the onset were 20-30 years, those of the death were 40-50 years. The latter was delayed about 20 years in comparison with a previous report. In the recent cases, the vascular lesions widely expanded. Luminal dilatation and aneurysm formation also increased in frequency, their ratio being approximately 57%. In the autopsy cases, the following active lesions were observed: (1) acute exudative inflammation (including suppuration), (2) chronic non-specific productive inflammation and (3) various types of granulomatous inflammation. These findings suggest that many triggers may play a role in the morphogenesis of Takayasu arteritis. The inflammatory lesions are produced in the media and adventitia through the vasa vasorum, and terminate in a diffuse or nodular fibrosis. New active lesions are often observed near the old fibrotic ones. This suggests that Takayasu arteritis may be a progressive disease. Intimal thickening of the peripheral branches from the affected arteries is very often observed. In consequence, secondary ischemic lesions are formed in various organs, especially the heart, brain and kidneys.

Takayasu arteritis is a disease on which many investigations have been conducted to determine its causes, clinical features and treatment, since 1908 when it was reported by Dr. Takayasu. The first nationwide epidemiological survey on Takayasu arteritis was conducted over 3 years from 1973 through 1975 by a research group on "aortitis syndrome", a disease specified by the Ministry of Health and Welfare. Another nationwide survey on this disease over a period of 3 years was carried out from 1982 through 1984 by a research group focusing on "systemic vascular lesion", another disease specified by the Ministry of Health and Welfare. The present study reports on the combined results of these two nationwide epidemiological surveys.

To characterize aortic regurgitation in patients with Takayasu arteritis, we studied 48 females with arteritis (mean age 47 +/- 12 years) by means of color Doppler echocardiography. Aortic regurgitation was confirmed in 32 out of 48 patients (67%) by color-flow mapping. Twenty-four patients had mild or no aortic regurgitation (group A), 9 had moderate (group B), and 15 had severe (group C) aortic regurgitation. We compared the echocardiographic data obtained from patients with Takayasu arteritis with those of 14 normal controls and 9 patients with severe aortic regurgitation of valvular origins (group V). The aortic root diameter (AOD) in group B (23 +/- 4 mm/M2) and group C (22 +/- 3 mm/M2) revealed a statistically significant large value as compared with that in group A (18 +/- 2 mm/M2) and normal controls (17 +/- 3 mm/M2). However, the differences, between groups B and C and groups C and V, were not significant. The AOD was not obviously dilated in a considerable number of group C patients. Aortic valve involvement was seen in several group C patients and moderate concentric left ventricular hypertrophy was present in all group C patients. Group C patients therefore, have concentric left ventricular hypertrophy but may or may not have dilatation of the aortic root which can be detected on echocardiography. We conclude that aortic valve involvement may cause aortic regurgitation in some patients with Takayasu arteritis and that aortic regurgitation is more common than previously believed.