Background

Kawasaki disease (KD) is a systemic vasculitis syndrome that occurs most frequently in children. Most clinical and pathological studies have focused on its coronary artery lesions. To date, no detailed studies of the aorta have been conducted. We studied KD autopsy cases with the aims of clarifying the time-course of changes in aortic lesions, the differences in the inflammatory cells and degree of inflammation at various aortic sites, and the progression of the inflammation.

Materials and Methods

The study materials were aortic specimens taken from 37 KD autopsy cases (acute phase: 19; remote phase: 18). Twenty-seven of the cases also had coronary aneurysms. We chose 3 aortic sites, i.e., the thoracic aorta, aortic root and aortic bifurcation, and we histologically observed and compared those sites in regard to the changes with time, the kinds of infiltrating cells and the number of inflammatory cells. We also observed the relationship between the vasa vasorum and inflammatory cell localization in the tunica media, and examined the progression of inflammation in the tunica media.

Results

Destruction of the vascular architecture was not seen in any of the 37 cases, but inflammatory cell infiltration was observed in 90% of the acute-phase cases. The inflammatory cell infiltration involved the tunica intima and tunica adventitia of the aorta on the 6th disease-day, and all layers of the aorta on the 13th disease-day; the infiltration peaked on the 18th disease-day. The infiltration gradually disappeared thereafter, and no significant infiltration was seen in the remote phase. The infiltrating inflammatory cells consisted mainly of CD163-positive macrophages. Comparison of the 3 sites of the aorta showed that the inflammatory cell infiltration was more severe in the aortic root and aortic bifurcation than in the thoracic aorta. The progression of inflammation to the aortic tunica media from the adventitia showed 2 patterns: 1 in which macrophages were aggregated around the vasa vasorum; and a second in which there was no such aggregation around the vasa vasorum, but there was diffuse inflammatory cell infiltration of the tunica media. In addition to this, there were findings of direct infiltration of cells from the tunica intima into the tunica media.

Conclusion

Inflammation in KD occurs in the aorta. The changes with time and the kinds of infiltrating cells were the same as reported to date for coronary arteries in KD. There were differences in the degree of inflammation among the 3 aortic sites. It can be thought that the inflammation from the adventitia to the media progresses via the vas vasorum, and also, there is a possibility of spreading directly. From the intima to the media, inflammation spreads directly. However, formation of aneurysms and destruction of the vascular architecture of the aorta were absent in this study, unlike in coronary arteries.

中文翻译：

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川崎病主动脉炎的病理分析：37例研究

背景

川崎病（KD）是一种系统性血管炎综合征，在儿童中最常见。大多数临床和病理研究都集中在其冠状动脉病变上。迄今为止，尚未对主动脉进行详细研究。我们研究了KD尸检病例，目的是弄清主动脉病变的变化的时程，各个主动脉部位的炎症细胞和炎症程度的差异以及炎症的进展。

材料和方法

研究材料是从37例KD尸检病例中提取的主动脉标本（急性期：19；远程期：18）。二十七例也有冠状动脉瘤。我们选择了3个主动脉部位，即胸主动脉，主动脉根和主动脉分叉部位，并在组织学上观察并比较了这些部位在时间，浸润细胞种类和炎性细胞数量方面的变化。我们还观察了脉管血管与中膜介质中炎性细胞定位之间的关系，并检查了中膜介质中炎症的进展。

结果

在这37例病例中，均未观察到血管结构破坏，但在90％的急性期病例中观察到炎症细胞浸润。炎性细胞浸润在第6个病日涉及主动脉的内膜和外膜外膜，在第13个病日涉及主动脉的所有层。在第18个病日的渗透达到高峰。此后渗透逐渐消失，并且在远期没有看到明显的渗透。浸润的炎性细胞主要由CD163阳性巨噬细胞组成。对三个主动脉部位的比较显示，炎性细胞浸润在主动脉根部和主动脉分叉处比在胸主动脉中更为严重。外膜从炎症到主动脉膜介质的进展显示出两种模式：1个，其中巨噬细胞聚集在血管脉管周围；第二个是血管脉管周围没有这种聚集，但是中膜弥漫性炎性细胞浸润。除此之外，还发现了细胞从内膜内膜直接渗入中膜培养基。

结论

KD炎症发生在主动脉中。随时间的变化和浸润细胞的种类与迄今为止报道的KD冠状动脉相同。3个主动脉部位的炎症程度存在差异。可以认为，从外膜到中膜的炎症通过血管扩张，并且有可能直接扩散。从内膜到中膜，炎症直接扩散。但是，与冠状动脉不同，本研究中没有形成动脉瘤和破坏主动脉的血管结构。