Boston Children's Hospital-Pediatric Cardiology, Harvard Medical School, Boston, Massachusetts, USA
Cardiology, Boston Children's Hospital, Boston, Massachusetts, USA.
Arch Dis Child. 2021 Mar;106(3):247-252. doi: 10.1136/archdischild-2020-319810. Epub 2020 Sep 17.
Patients with Kawasaki disease (KD) with coronary artery enlargement at diagnosis are at the highest risk for persistent coronary artery aneurysms (CAAs) and may benefit from primary adjunctive anti-inflammatory therapy beyond intravenous immunoglobulin (IVIG). We evaluate the effect of primary adjunctive corticosteroid therapy on outcomes in patients with CAA at diagnosis.
Single-centre, retrospective review.
Patients with KD diagnosed within 10 days of fever onset and with baseline CA z-score ≥2.5.
Primary treatment with IVIG (n=162) versus IVIG plus corticosteroids (n=48).
Treatment resistance (persistent fever >36 hours after initial treatment), CAA regression rate.
Of the 92 patients with KD who received corticosteroids at our institution from 2012 to 2019, 48 met the inclusion criteria for primary adjunctive therapy. The corticosteroid group was younger and had larger baseline CAAs compared with historical controls. Demographics and laboratory values were otherwise similar between groups. The corticosteroid group had a less treatment resistance (4% vs 30%, p=0.003) and a greater improvement in C reactive protein. After adjusting for baseline CA z-score, age and baseline bilateral versus unilateral CAA, the corticosteroid group had a higher odds of (OR 2.77 (1.04, 7.42), p=0.042) and a shorter time to CAA regression (HR 1.94 (1.27, 2.96), p=0.002).
Primary adjunctive corticosteroid therapy is associated with decreased initial treatment resistance, greater improvement in inflammatory markers and higher likelihood of CAA regression in patients who have CAA at diagnosis. Multi-centre, randomised controlled trials are needed to confirm the benefits of corticosteroids in patients with CAA at diagnosis and to compare corticosteroids with other adjunctive therapies.
在诊断时伴有冠状动脉扩张的川崎病（KD）患者发生持续性冠状动脉瘤（CAA）的风险最高，可能受益于静脉注射免疫球蛋白（IVIG）以外的主要辅助抗炎治疗。我们评估在诊断时即存在 CAA 的患者中，初始辅助皮质类固醇治疗对结局的影响。
单中心回顾性研究。
在发热发病后 10 天内诊断为 KD 的患者，且基线冠状动脉 z 评分≥2.5。
初始治疗采用 IVIG（n=162）或 IVIG 加皮质类固醇（n=48）。
治疗抵抗（初始治疗后持续发热超过 36 小时），CAA 消退率。
在我们机构 2012 年至 2019 年接受皮质类固醇治疗的 92 例 KD 患者中，有 48 例符合初始辅助治疗的纳入标准。皮质类固醇组年龄较小，且基线时 CAA 更大。两组之间的人口统计学和实验室值均相似。皮质类固醇组的治疗抵抗发生率较低（4%比 30%，p=0.003），C 反应蛋白改善更明显。在校正基线冠状动脉 z 评分、年龄以及基线双侧与单侧 CAA 后，皮质类固醇组 CAA 消退的可能性更高（OR 2.77（1.04，7.42），p=0.042），消退时间更短（HR 1.94（1.27，2.96），p=0.002）。
在诊断时即存在 CAA 的患者中，初始辅助皮质类固醇治疗与初始治疗抵抗降低、炎症标志物改善更明显以及 CAA 消退可能性更高相关。需要进行多中心、随机对照试验以证实皮质类固醇在诊断时存在 CAA 的患者中的获益，并比较皮质类固醇与其他辅助治疗。
