Department of Clinical Immunology and Rheumatology, Sanjay Gandhi Postgraduate Institute of Medical Sciences, Lucknow, 226014, India.
Clin Rheumatol. 2023 Oct;42(10):2703-2715. doi: 10.1007/s10067-023-06621-9. Epub 2023 May 9.
Cardiovascular involvement in juvenile rheumatic diseases is the primary manifestation in paediatric vasculitis and a major organ manifestation in paediatric connective tissue diseases. Though coronary vasculitis is the prototypical manifestation of Kawasaki disease, it can also be seen in patients with polyarteritis nodosa. Pericarditis is the most common manifestation seen in juvenile rheumatic diseases like systemic onset JIA, and lupus. Cardiac tamponade, valvular insufficiency, aortic root dilatation and arrhythmias are seen rarely. Cardiac involvement is often recognized late in children. The development of cardiac disease in juvenile systemic sclerosis is associated with a poor outcome. In long term, childhood onset of rheumatic diseases predisposes to diastolic dysfunction and premature atherosclerosis during adulthood. Key Points • Pericarditis is the most common cardiac manifestation in SLE and can lead to tamponade. • Conduction defects are common in juvenile mixed connective tissue disease and systemic sclerosis. • Pulmonary hypertension is a significant contributor to mortality in juvenile systemic sclerosis. • In Kawasaki disease, early treatment can reduce risk of coronary artery aneurysms.
心血管受累是儿童血管炎的主要表现，也是儿童结缔组织病的主要器官表现。虽然冠状动脉血管炎是川崎病的典型表现，但也可见于结节性多动脉炎患者。心包炎是全身型幼年特发性关节炎和狼疮等幼年风湿性疾病中最常见的表现。心脏压塞、瓣膜功能不全、主动脉根部扩张和心律失常则较为少见。心脏受累在儿童中往往被发现较晚。儿童发生心脏疾病与幼年系统性硬化症的不良预后相关。从长远来看，幼年起病的风湿性疾病会导致成年期舒张功能障碍和早发性动脉粥样硬化。
关键点
心包炎是系统性红斑狼疮中最常见的心脏表现，并可导致心脏压塞。
传导障碍在幼年混合性结缔组织病和系统性硬化症中很常见。
肺动脉高压是幼年系统性硬化症患者死亡的重要原因。
在川崎病中，早期治疗可以降低冠状动脉瘤的风险。
