Detection of coronary artery aneurysms,stenoses and occlusions by multislice spiral computed tomography in adolescents with kawasaki disease

In patients with Kawasaki disease (KD), serial evaluation of coronary artery aneurysms (CAAs) and luminal narrowing is essential for risk stratification and therapeutic management. Therefore, non-invasive assessment of the status of the coronary artery is of utmost importance in patient management. Multislice spiral computed tomography (MSCT) permits non-invasive visualization of the entire coronary artery system and was used in the evaluation of 4 patients with KD. CAAs and high-grade coronary artery stenoses were detected by MSCT and corroborated the findings of coronary angiograms performed within the previous 2 years. MSCT has the potential to be the standard diagnostic tool in adolescents with KD.     

Treatment of coronary aneurysms with covered stents: a review with illustrated case

Coronary aneurysms are found in approximately 5% of patients undergoing coronary angiography. Most coronary artery aneurysms (CAAs) are associated with significant stenosis of the coronary lumen; however, there is no common consensus on the treatment of coronary aneurysms. A large aneurysm in the coronary artery makes the blood flow turbulent and predisposes to thrombus formation and coronary artery obstruction even without the presence of significant stenosis. Despite this important anatomical abnormality of the coronary artery, the treatment options are poorly understood and present a therapeutic challenge to the interventional cardiologist. While treating CAAs with percutaneous implantation of covered stents offers a less invasive option compared to surgical correction, the short- and long-term outcomes are unknown. In this article, we have reviewed the literature and discussed the pathophysiology, clinical importance, and treatment options for CAAs. The illustrated case demonstrates a difficult situation in the management of CAA with interesting images, including coronary angiography, intravascular ultrasound, and transesophageal echocardiography of the complication encountered in this particular case.     

冠状动脉扩张病流体力学观测及其初步应用效果

目的 研究冠状动脉瘤样扩张、冠状动脉弥漫扩张在临床表现、造影特征及血液动力学方面的区别,并尝试利用流体力学分析结果指导冠状动脉扩张病的治疗。 方法 回顾性筛查了连续22,456份冠脉造影资料及相关患者的临床资料。利用SimVacular软件,探究了冠状动脉扩张病中的血液流体力学变化并辅助临床患者治疗。 结果 冠状动脉扩张病总体发病率为1.25 %,与冠状动脉瘤样扩张组相比,冠状动脉弥漫扩张患者中男性比例明显升高(P<0.01),年龄更加年轻（P<0.01）,且吸烟患者比例较少（P<0.05）。冠状动脉瘤样扩张在冠脉上的分布情况有别于冠状动脉弥漫扩张(P<0.01),且冠状动脉瘤样扩张更易合并冠脉狭窄(P<0.01)。平均壁面剪切力在冠状动脉瘤样扩张病变中明显下降,伴剪切震荡因子明显升高。随着冠状动脉瘤样扩张程度增大,平均壁面剪切力和剪切震荡因子的变化更加明显,冠脉内出现涡流。随着冠脉瘤样扩张形态由梭形趋近于球形,冠脉内血流出现紊乱,且流体力学的变化主要发生于扩张的起始端。根据流体力学分析结果指导冠状动脉扩张病患者的治疗,其中10名患者免于介入治疗,两年后随访,6名患者生存状况良好,无不良反应发生,3名患者失访,1名患者死于未知原因。 结论 冠状动脉瘤样扩张、冠状动脉弥漫扩张在临床表现、造影特征及血液动力学方面具有不同的表现特征,其中冠状动脉瘤样扩张更倾向于是一种冠状动脉粥样硬化的特殊类型,而冠状动脉弥漫扩张更倾向于是一种免疫系统疾病。利用血液流体力学分析结果可为临床冠状动脉扩张病患者的治疗提供定量依据。     

Congenital and atherosclerotic (acquired) coronary artery aneurysms: Coronary angiographic and morphologic observations in 10 patients

Coronary angiographic observations in 10 patients with coronary artery aneurysms (CAAs) are reported. Four patients had atherosclerotic (acquired) and six had congenital CAAs. The mean age of patients with acquired CAAs was higher (64.7 years) compared with the congenital group (53.2 years). Ipsilateral myocardial infarction (MI) occurred in three of four patients with acquired CAAs but MI was not located on the same side as the aneurysm-bearing coronary artery (contralateral) in two patients with the congenital variety. In the patients with congenital CAAs, an aneurysm predilection site was observed in the proximal portion of the aneurysm-bearing vessel. Dual and multiple aneurysms were more common in the acquired CAAs. Although congenital CAAs were sizeable, the small-sized atherosclerotic CAAs developed complications more frequently. During an average follow-up of 7.9 years, only one patient died of a noncardiac cause and another developed recurrent uncomplicated non-Q wave lateral MI. Both subjects had atherosclerotic CAAs. In our series, no rupture or sudden death occurred. Coronary artery bypass grafting (CABG) was performed in three patients with acquired and in one patient with congenital CAAs. In the latter patient, simultaneous ligation of the congenital aneurysm associated with a coronary arteriovenous fistula was performed. Regarding antiplatelet and anticoagulant policy for the whole group, three patients were on aspirin, four were on acenocoumarol, and in another three subjects with congenital CAAs, a medical regimen was followed. Larger series, however, are required in order to elucidate further angiographic characteristics of acquired vs congenital CAAs.     

Kawasaki disease: a maturational defect in immune responsiveness

Kawasaki disease (KD), an acute febrile disease in children of unknown etiology, is characterized by a vasculitis that may result in coronary artery aneurysms (CAAs). In new patients with KD, a selective and prolonged T cell unresponsiveness to activation via the T cell antigen receptor CD3 was observed, whereas proliferation to other stimuli was intact. This "split T cell anergy" delineated KD from other pediatric infections and autoimmune diseases and correlated with CAA formation (P<.001). A transient immune dysfunction was also suggested by an incomplete responsiveness to measles-mumps-rubella (MMR) vaccination in patients with KD versus controls (P<.0001; odds ratio, 15.6; 95% confidence interval, 4.8-51.1), which was overcome by revaccination(s). The reduced responsiveness to MMR in patients with KD suggests a subtle and predetermining immune dysfunction. An inherent immaturity to clear certain antigens may be an important cause that precipitates KD and the immune dysregulation during acute disease.     

Mid-term follow-up of coronary artery aneurysm after directional coronary atherectomy

Coronary artery aneurysm (CAA) occurs in 6-12% of lesions after directional coronary atherectomy (DCA). The prognosis and the optimal treatment for DCA-related CAAs have not been well known. Therefore, we reviewed the clinical course of 214 consecutive patients with DCA-related CAAs who underwent DCA in our hospital. Follow-up coronary angiography 6 months after DCA was completed in 193 patients (212 lesions) and 14 lesions with CAAs (14 patients) were detected. We evaluated these 14 lesions by repeat coronary angiography at an average of 32 months after DCA in comparison with the adjacent reference vessel. Twelve of the 14 patients have been uneventful but 2 suffered from de novo angina due to new stenotic lesion unrelated to the DCA procedures. We compared the preprocedural angiographic characteristics and periprocedural parameters between the 14 lesions with CAAs[CAA(+)group] and the 198 without CAAs [CAA(-)group], but found no significant differences. Histological examination of specimens retrieved during atherectomy demonstrated that subintimal resection was more frequent in the CAA(+)group(57%) than the CAA(-)group(31%). The diameter of the aneurysm divided by the reference diameter was significantly larger at 6 months immediately after DCA(1.71 +/- 0.21 vs 1.31 +/- 0.18, p < 0.05) but did not change subsequently (1.68 +/- 0.23). Our retrospective analysis revealed a good mid-term (an average of 32 months) prognosis for CAAs found by routine follow-up coronary angiography and also demonstrated that the depth of resection was significantly associated with aneurysm formation.     

Two adult cases of coronary artery aneurysms secondary to Kawasaki disease

Kawasaki disease (KD) is an acute, self-limiting vasculitis that occurs in children of all ages, which was first described by Kawasaki in 1967. The fatal complication of KD is coronary artery involvement resulting in coronary artery aneurysms. We report on two adult cases with coronary artery aneurysms secondary to childhood KD who underwent coronary artery bypass grafting (CABG) using multiple arterial grafts. There have been few reports of cardiovascular surgery in adult survivors of KD.     

Sensitivity, specificity and predictive value of two-dimensional echocardiography in detecting coronary artery aneurysms in patients with Kawasaki disease

Seventy-seven patients with Kawasaki disease were prospectively evaluated from 1978 to 1983 to determine the sensitivity, specificity and predictive value for detecting coronary artery aneurysms with two-dimensional echocardiography. Seventy (91%) underwent selective coronary arteriography and are included in this report. The study was divided into two periods because of increased experience and the use of a systematic approach with two-dimensional echocardiography in the second period as compared with the first. Aneurysms were demonstrated in nine patients (13%). The coronary artery system was divided into six regions: proximal third of the main right coronary artery, distal right coronary artery, left main coronary artery, left anterior descending coronary artery, circumflex coronary artery and distal left coronary artery. The sensitivity and specificity were high when imaging the proximal regions, and improved from the first period to the second. Both sensitivity and specificity were lower for the more distal regions of the right and left coronary arteries. Overall, the sensitivity of two-dimensional echocardiography was 100% because there were no patients in our study who had isolated distal coronary artery aneurysms. Two-dimensional echocardiography is a sensitive and specific test for detecting aneurysms in the proximal portions of both the right and left coronary arteries, and is useful in selecting patients for invasive investigation with selective coronary arteriography.     

Two-dimensional echocardiographic analysis of proximal left main coronary artery in humans

The left main coronary artery (LMCA) was evaluated in 100 consecutive patients (88 men and 12 women; mean age 63 years) with anginal syndrome, all in New York Heart Association classes II and III. Each patient underwent two-dimensional echocardiography (2DE) from the parasternal short-axis and apical four-chamber views. Coronary angiography was subsequently performed within 24 hours. The LMCA was directly measured by 2DE and coronary angiography at its widest point. Each echocardiogram was blindly evaluated for LMCA aneurysm or obstruction. Eight patients (8%) were excluded because of inadequate visualization of the LMCA. The mean 2DE measurement was 4.4 +/- 0.9 mm vs 4.2 +/- 0.8 mm on coronary angiography (r = 0.86). Atherosclerotic aneurysms of the LMCA were correctly diagnosed in two patients by 2DE. LMCA stenosis (greater than 50%) was found in 11 patients on coronary angiography; three of them had ostial or proximal lesions, three had middle lesions, and five had distal lesions. 2DE correctly diagnosed all three ostial lesions, two of three middle lesions, but only two of five distal lesions. In four patients, dense echoes in the LMCA caused a false positive diagnosis. It was concluded that: the LMCA can be visualized and correctly measured by 2DE; atherosclerotic aneurysms can be detected; and 2DE is yet unable to screen patients for LMCA lesions; however, 2DE is a promising method for evaluating proximal and especially ostial LMCA stenosis.     

Validation of a clinical-significance-based classification of coronary artery anomalies

The clinical significance and subsequent management of the various types of coronary artery anomalies (CAAs) are different, as is the relationship to sudden death, coronary artery disease, and myocardial ischemia. A practical global classification based on clinical significance has not yet been proposed. This retrospective study was aimed at evaluating the current clinical significance of CAAs and the effectiveness of a clinical-significance-based classification. In a single-center retrospective study at a public hospital, a review of the last 5,100 coronary angiographies was performed in order to select patients with CAAs. The CAAs were classified into 4 categories on the basis of a literature review according to angiographic appearance and clinical significance: benign (class I); relevant-associated with fixed myocardial ischemia (class II); severe-related to sudden death (class III); and critical-associated with superimposed coronary artery disease (CAD) (class IV). Clinical and instrumental records of the selected patients were reviewed as well as the occurrence of cardiovascular events from the date of diagnosis to July 2002. Sixty-two patients (1.2%, women/men 20/42, mean age 65.3 +/-10.6 years) had CAA on coronary angiography. From the above-described classification, 40 patients were categorized in class I (64.5%), 9 in class II (14.5%), 7 in class III (11.3%), and 6 in class IV (9.7%). During the follow-up (mean duration 60.4 +/-12.3 months) most cardiovascular events and death occurred in class III and IV patients (71.3% and 100% respectively). A high correlation was found between significance level and percentage of patients with cardiovascular events (r = 0.99). Actuarial survival at 5 years was 82.9%. CAAs can be practically classified on the basis of clinical presentation and significance. This clinical classification may be useful in managing patients with CAA and personalizing their follow-up and therapeutic options according to their class and case histories.     

川崎病合并冠状动脉损害的临床超声随访简

目的 探讨川崎病（Kawasaki disease,KD）合并冠状动脉损害患儿的临床预后.方法 回顾分析2002年1月至2011年12月收治的KD合并冠状动脉损害254例患儿的临床诊治与随访资料.结果 本组冠状动脉损害占KD患者总数的28％,其中冠状动脉扩张（CAD）216例,占KD的28.16％,冠状动脉瘤（CAA）38例,占4.2％.随访结果：随访冠状动脉扩张210例,其中有单侧（左或右冠状动脉）冠状动脉扩张99例（47.14％）随访3个月内恢复正常;21例（17.5％）6～12个月内恢复正常.双侧（左、右冠状动脉）冠状动脉扩张26例（11.26％）随访12～18个月恢复正常,59例（25.54％）随访18～36个月恢复正常,5例（近期）仍在随访中.随访冠状动脉瘤35例,冠状动脉瘤内径＜6 mm 28例12～36个月恢复正常,冠状动脉内径＞8 mm的巨大动脉瘤7例,2例随访12～24个月尚未恢复,3例随访24～36个月双侧均有回缩或单侧恢复后留有不同程度的冠状动脉扩张;2例分别随访6年、8年仍存单侧冠状动脉瘤,另侧冠状动脉扩张.结论 轻、中度冠状动脉扩张预后较好,冠状动脉瘤的恢复相对冠状动脉扩张时间长;巨大动脉瘤特别伴有心肌病变,以及多脏器损害预后较差.     

Comprehensive Evaluation of a Patient with Kawasaki Disease and Giant Coronary Aneurysms with Cardiac Magnetic Resonance

In patients with Kawasaki disease (KD) and coronary artery aneurysms (CAAs), serial evaluation with multiple imaging modalities is recommended to guide risk stratification and management. We present the case of a 7‐year‐old boy with a history of KD and multiple giant CAAs who developed thrombosis in one of the aneurysms. We illustrate how cardiac magnetic resonance is unique in allowing a comprehensive assessment in patients with severe KD and how it may affect their management.     

Kawasaki disease, myocardial infarction and coronary artery revascularization

Kawasaki disease (KD) is the leading cause of acquired pediatric heart disease in North America and Japan. Cardiac sequelae, such as coronary artery aneurysms and myocardial infarction, are the major causes of the morbidity and mortality associated with KD. Three case scenarios are described illustrating the wide range of clinical presentations of myocardial ischemia in children after acute KD, varying from asymptomatic to fatal myocardial infarction. In addition, the present paper provides a review of the literature on myocardial infarction in association with KD and various modalities of coronary artery revascularization in children with myocardial ischemia secondary to KD.     

Ultrastructural characteristics of myocardial and coronary microvascular lesions in Kawasaki disease.

Kawasaki disease (KD) is an acute febrile mucocutaneous lymph node syndrome with multisystemic vasculitis affecting mainly infants and children. Although several studies on cardiovascular lesions in KD have been done at the light microscopic level, the ultrastructural characteristics and incidences of these lesions have not been well defined. In order to investigate the myocardial and coronary microvascular sequelae in KD, we performed an ultrastructural study on endomyocardial biopsy specimens obtained during follow-up from 54 patients who had typical clinical manifestations of KD, of whom 47 had associated coronary aneurysms as demonstrated by coronary arteriography (CAG) or two-dimensional echocardiography in the acute or healed stage. The average age of onset was 2.2 years old and the duration of illness was from 2 months to 23 years. Follow-up CAG showed that the coronary aneurysms persisted in 33 of the 47 patients (8 with associated stenosis) and resolved in the remaining 14 patients. Ultrastructurally, the myocardial changes revealed hypertrophy, various degrees of degeneration, proliferation and abnormality of mitochondria, infiltration of a small number of lymphocytes, and fibrosis. The coronary microvascular lesion was characterized by microvascular dilatation, endothelial cell injury, platelet aggregation with thrombosis, and stenotic lumen with thickened walls in the small arterioles. It persisted after convalescent stage even up to 23 years and closely correlated with the myocardial sequelae. Moreover, significantly increased incidences of myocardial and coronary microvascular lesions were found in patients with coronary artery lesion. These findings suggest the coronary microvascular lesion as a possible underlying factor of persistent sequelae in KD.     

The older patient with syncope: practicalities and controversies

Introduction

Corticosteroid administration in Kawasaki disease (KD) is controversial but accepted as treatment for patients who do not respond to initial treatment. The impact of corticosteroids on evolving coronary artery aneurysms (CAA) and future vascular remodelling is unknown.

Methods and results

The clinical history of 80 patients (73% male; median age at diagnosis 2.2 years) seen from 1990 to 2008 with CAAs after KD were reviewed, 19 (24%) of whom received systemic corticosteroids in the acute phase (14 for ≤ 3 days, 5 for 4+ days). CAA z-scores were assessed at baseline, 2-3 months, and 1 year after the acute phase. Linear regression models adjusted for repeated measures were used to determine the association between change in CAA z-score over time and corticosteroid use, adjusting for patient age at diagnosis, gender, intravenous immunoglobulin use, total days of fever, albumin level, hemoglobin level and platelet count.

Results

The corticosteroid treated group had longer duration of fever in the acute phase (median 17 vs. 11 days, p = 0.04). Adjusted CAA z-scores at diagnosis, 2-3 months and 1 year follow-up for CAA in the left anterior descending decreased (from + 5.5 to + 3.5 to + 1.9) in those not treated with corticosteroids, but progressed for those treated with corticosteroids (from + 7.4 to + 17.5 to + 15.8), regardless of duration of corticosteroid treatment. Similar results were noted for CAA of the right coronary artery and the left main coronary artery.

Conclusions

The use of corticosteroids in the acute phase of KD for patients with evolving CAAs may be associated with worsening involvement and impaired vascular remodelling and warrants further study.     

Low-Molecular-Weight Heparin vs Warfarin for Thromboprophylaxis in Children With Coronary Artery Aneurysms After Kawasaki Disease: A Pragmatic Registry Trial

BackgroundThe substantial risk of thrombosis in large coronary artery aneurysms (CAAs) (maximum z-score ≥ 10) after Kawasaki disease (KD) mandates effective thromboprophylaxis. We sought to determine the effectiveness of anticoagulation (low-molecular-weight heparin [LMWH] or warfarin) for thromboprophylaxis in large CAAs.MethodsData from 383 patients enrolled in the International KD Registry (IKDR) were used. Time-to-event analysis was used to account for differences in treatment duration and follow-up.ResultsFrom diagnosis onward (96% received acetylsalicylic acid concomitantly), 114 patients received LMWH (median duration 6.2 months, interquartile range [IQR] 2.5-12.7), 80 warfarin (median duration 2.2 years, IQR 0.9-7.1), and 189 no anticoagulation. Cumulative incidence of coronary artery thrombosis with LMWH was 5.7 ± 3.0%, with warfarin 6.7 ± 3.7%, and with no anticoagulation 20.6 ± 3.0% (P < 0.001) at 2.5 years after the start of thromboprophylaxis (LMWH vs warfarin HR 1.5, 95% confidence interval [CI] 0.4-5.1; P = 0.56). A total of 51/63 patients with coronary artery thrombosis received secondary thromboprophylaxis (ie, thromboprophylaxis after a previous thrombus): 27 LMWH, 24 warfarin. There were no differences in incidence of further coronary artery thrombosis between strategies (HR 2.9, 95% CI 0.6-13.5; P = 0.19). Severe bleeding complications were generally rare (1.6 events per 100 patient-years) and were noted equally for patients on LMWH and warfarin (HR 2.3, 95% CI 0.6-8.9; P = 0.25).ConclusionsLMWH and warfarin appear to have equivalent effectiveness for preventing thrombosis in large CAAs after KD, although event rates for secondary thromboprophylaxis and safety outcomes were low. Based on our findings, all patients with CAA z-score ≥ 10 should receive anticoagulation, but the choice of agent might be informed by secondary risk factors and patient preferences.     

三磷酸腺苷负荷超声心动图和冠状动脉造影在川崎病远期随访中的价值

目的探讨三磷酸腺苷（adenosinetriphosphate，ATP）负荷超声心动图及冠状动脉造影（coronaryangiography，CAG）在川崎病（Kawasakidisease，KD）冠状动脉损害远期随访中的应用价值。方法对临床确诊的156例KD患儿于恢复期行ATP负荷超声心动图检查，其中15例患儿同时进行CAG。随访时间为自急性期后6个月至10年，按急性期冠状动脉损害程度分为冠状动脉瘤组、冠状动脉扩张组及冠状动脉正常组。结果恢复期ATP负荷超声检查结果显示，3组均有部分患儿存在不同程度的冠状动脉血流储备下降和（或）出现节段性室壁运动异常，其在冠状动脉瘤组和冠状动脉扩张组的发生率高于冠状动脉正常组，差异有统计学意义[75．00％（30／40）vs.44．29％（31／70）vs．21．74％（10／46），P〉0．01]。15例同时进行CAG与负荷超声检查的患者结果显示，两种检查方法的符合率为93．33％（Kappa=0．04，P=0．70）。结论CAG能较清楚地显示冠状动脉形态，但不能准确地提供冠状动脉血流动力学信息及冠状动脉微循环的情况；ATP负荷超声心动图检查能安全、可靠地评估冠状动脉血流动力学，补充冠状动脉造影的不足，可用于KD患儿冠状动脉损害的长期追踪随访以及药物疗效的判断。     

CD8 T lymphocytes and macrophages infiltrate coronary artery aneurysms in acute Kawasaki disease

The pathogenesis of coronary arterial inflammation in acute Kawasaki disease (KD) is unclear. To test the hypothesis that the KD vascular lesion is an activated T lymphocyte-dependent process, immunohistochemical studies were done on coronary artery aneurysms from 8 fatal acute KD cases by using antibodies to CD45RO (activated or memory T lymphocyte), CD8 (cytotoxic T lymphocyte), CD4 (helper T lymphocyte), HAM56 (macrophage), and CD20 (B lymphocyte). Acute KD coronary arteritis was characterized by transmural infiltration of CD45RO T lymphocytes with CD8 T lymphocytes predominating over CD4 T lymphocytes. Macrophages were present primarily in the adventitial layer; B lymphocytes were notably absent. These data lend support to the hypotheses that KD results from infection with an intracellular pathogen, such as a virus, whose antigens are presented by major histocompatibility complex class I molecules, and that CD8 T lymphocytes and macrophages are important in the pathogenesis of KD coronary aneurysms.     

Changes in the coronary arteries in Kawasaki disease: echocardiographic aspects

OBJECTIVE: Evaluation of coronary anomalies in children with Kawasaki disease (KD) by two-dimensional echocardiography. SETTING: Outpatient and Laboratory of Echocardiography in the Pediatric Cardiology Department of Santa Marta Hospital. PATIENTS: Forty four children (M/F = 1.7/1) with a mean age of 2 years and 8 months (range from 2 months to 8 years) underwent Outpatient and serial echocardiographic study from July 1984 to December 1988. RESULTS: Coronary artery anomalies were demonstrated in 13 (52%) of 44 children; 11 (25%) had uniform dilatation and 12 (27%) had aneurysms. These anomalies were never observed before the first week of illness. During a mean follow-up of 8.4 months, we have observed normalization of coronary arteries lumen, in 10 (43.4%) of the 23 cases. The anomalies remained in 13 (56.6%). CONCLUSIONS: Coronary artery anomalies are a frequent complication of KD. In about 50% of patients, coronary artery anomalies remained in a mean follow-up of 8 months. Two-dimensional echocardiography is a good method to study coronary anomalies in KD.     

HLA–E gene polymorphism associated with susceptibility to kawasaki disease and formation of coronary artery aneurysms

Objective

Kawasaki disease (KD) is a pediatric systemic vasculitis of unknown cause for which a genetic influence is supposed. The purpose of this study was to identify possible genetic variants in the major histocompatibility complex (MHC) region that are associated with KD and the development of coronary artery aneurysms (CAAs) in a Taiwanese population.

Methods

The 168 genetic variants covering the MHC locus were analyzed in an association study of a Taiwanese cohort of 93 KD patients and 680 unrelated healthy children matched for sex and age with the study patients.

Results

Eleven single‐nucleotide polymorphisms (SNPs) were associated with the occurrence of KD. The SNP located at the 3′‐untranslated region of HLA–E (rs2844724) was highly associated (P < 1 × 10⁻⁷). In addition, the frequency of the C allele was higher in KD patients without CAAs than in controls (P < 0.001) due to a significantly increased frequency of the CC and CT genotypes. Plasma levels of soluble HLA–E were significantly higher in KD patients than in controls regardless of the presence of CAAs. Furthermore, there was a trend toward higher plasma levels of soluble HLA–E in KD patients with the CT and TT genotypes of the HLA–E gene polymorphism.

Conclusion

Our results suggest that the HLA–E gene polymorphism may play a role in the pathogenesis of KD.