Suzuki A, Miyagawa-Tomita S, Nakazawa M, Yutani C
Department of Pediatrics, Tokyo Teishin Hospital, Japan.
Jpn Heart J. 2000 May;41(3):245-56. doi: 10.1536/jhj.41.245.
Since the original report of Kawasaki disease in 1967 more than 150,000 cases have been reported in Japan. Although there have been no nationwide epidemics in Japan since 1987, more than 6,000 newly diagnosed cases are reported every year, and the number has been increasing year by year despite the decreasing birth rate. The etiology of the disease is still unknown. High dose intravenous gammaglobulin is currently used during the acute phase in 84% of the patients in Japan with a concomitant decrease in coronary arterial sequelae. However, 7-13% of the patients still have persistent coronary artery aneurysms after the acute stage. The aneurysms are seen mostly in the proximal coronary arteries, and are often associated with aneurysms in the distal coronary artery segments (Figure 1A, 2A). Most of the patients show a decrease in the size of aneurysms soon after the acute phase (Figure 1B). However, the aneurysms may progress to obstructive lesions even after initial regression (Figures 1C, D, 2B). Such obstructive lesions may cause sudden death or myocardial infarction. Long term follow-up of coronary artery lesions has revealed several characteristic features, including progressive localized stenosis (Figure 1D), extensive recanalizations (Figure 2D) and development of collateral arteries. Progressive increases in aneurysm size and the appearance of new aneurysms in the late phase have also been reported. The basic mechanisms of the coronary arterial remodeling in Kawasaki disease have not yet been elucidated. Only recently has immunohistochemical staining in formalin-fixed specimens become feasible. This is a major technical breakthrough since it is almost impossible to obtain fresh frozen specimens of coronary artery lesions of Kawasaki discase. In this paper, we compare immunohistochemical findings in coronary artery lesions with the corresponding coronary angiographic findings, and attempt to make inferences as to the mechanism of remodeling both in early and late phases of the disease based on the expression of vascular growth factors.
自1967年首次报告川崎病以来，日本已报告超过15万例病例。尽管自1987年以来日本没有全国性的流行，但每年仍有超过6000例新诊断病例报告，而且尽管出生率下降，但病例数却逐年增加。该病的病因仍然不明。目前，日本84%的患者在急性期使用大剂量静脉注射丙种球蛋白，同时冠状动脉后遗症有所减少。然而，7% - 13%的患者在急性期后仍有持续性冠状动脉瘤。动脉瘤大多见于冠状动脉近端，且常与冠状动脉远端节段的动脉瘤相关（图1A、2A）。大多数患者在急性期后不久动脉瘤大小会减小（图1B）。然而，即使最初有所消退，动脉瘤仍可能进展为阻塞性病变（图1C、D、2B）。这种阻塞性病变可能导致猝死或心肌梗死。对冠状动脉病变的长期随访揭示了几个特征，包括进行性局部狭窄（图1D）、广泛再通（图2D）和侧支动脉形成。也有报道称晚期动脉瘤大小逐渐增加以及出现新的动脉瘤。川崎病冠状动脉重塑的基本机制尚未阐明。直到最近，在福尔马林固定标本中进行免疫组织化学染色才变得可行。这是一项重大的技术突破，因为几乎不可能获得川崎病冠状动脉病变的新鲜冷冻标本。在本文中，我们将冠状动脉病变的免疫组织化学结果与相应的冠状动脉造影结果进行比较，并试图根据血管生长因子的表达推断疾病早期和晚期重塑的机制。
