Falcini F, Trapani S, Turchini S, Farsi A, Ermini M, Keser G, Khamashta M A, Hughes G R
Department of Paediatrics, University of Florence, Italy.
Clin Exp Rheumatol. 1997 Nov-Dec;15(6):685-9.
Multiple humoral and cellular abnormalities in Kawasaki disease (KD) have already been described. In this study an analysis of immunological findings in a cohort of 34 Italian children affected with KD is reported, and the potential clinical significance of such alterations in predicting the development of coronary aneurysm and the prognosis of the disease is evaluated.
Levels of circulating immune complexes (CIC), antinuclear antibodies (ANA), anticardiolipin (aCL), antineutrophil cytoplasmic antibodies (ANCA), and anti-endothelial cells (AECA) and the T cell profile were determined in both the acute and the convalescent phases, and were compared to febrile, sex- and age-matched children.
CIC were present in 66% of the patients, 18 of whom were in the acute phase and 13 in the convalescent phase. In the control group CIC were detected in 47% of the children. ANA were negative in both the KD and in the febrile group. ANCA were present in 8%, AECA in 26%, and aCL in 30% of KD patients (IgG aCL antibodies were found in 14 patients, IgM aCL in, 1 and 1 had both). Among the controls, aCL antibodies were found in 5 patients (22%); in particular 1 (4.4%) had IgG and 4 (17.4%) had IgM aCL. An altered T cell profile, with an inverted CD4/CD8 ratio, was found in all KD children. All of the immune alterations showed a lower incidence in the convalescent than in the acute phase. No significant relationship between any of these immune findings and cardiac involvement or any other clinical manifestations was found.
Our data confirms the previously reported immunological anomalies in KD both in the acute and the convalescent phases, with a decreased incidence of such alterations in the convalescent phase. No prognostic significance for the occurrence of aneurysm could be demonstrated.
川崎病（KD）中已描述了多种体液和细胞异常。本研究报告了对34名患KD的意大利儿童队列的免疫学结果分析，并评估了这些改变在预测冠状动脉瘤发展和疾病预后方面的潜在临床意义。
在急性期和恢复期测定循环免疫复合物（CIC）、抗核抗体（ANA）、抗心磷脂（aCL）、抗中性粒细胞胞浆抗体（ANCA）、抗内皮细胞（AECA）水平以及T细胞谱，并与发热、性别和年龄匹配的儿童进行比较。
66%的患者存在CIC，其中18例处于急性期，13例处于恢复期。对照组中47%的儿童检测到CIC。KD组和发热组的ANA均为阴性。8%的KD患者存在ANCA，26%存在AECA，30%存在aCL（14例患者发现IgG aCL抗体，1例发现IgM aCL，1例两者均有）。对照组中，5例患者（22%）发现aCL抗体；特别是1例（4.4%）有IgG，4例（17.4%）有IgM aCL。所有KD儿童均发现T细胞谱改变，CD4/CD8比值倒置。所有免疫改变在恢复期的发生率均低于急性期。未发现这些免疫结果与心脏受累或任何其他临床表现之间存在显著关系。
我们的数据证实了先前报道的KD在急性期和恢复期的免疫异常，恢复期此类改变的发生率降低。未证明对动脉瘤发生有预后意义。
