Tselios Konstantinos, Urowitz Murray B
Centre for Prognosis Studies in the Rheumatic Diseases, Toronto Lupus Clinic, University Health Network, Toronto, Ontario. Canada.
Curr Rheumatol Rev. 2017;13(3):206-218. doi: 10.2174/1573397113666170704102444.
Systemic lupus erythematosus (SLE) is characterized by various clinical manifestations and immunologic abnormalities. Cardiovascular and respiratory system involvement are increasingly recognized as critical for patients' prognosis. In this review, current knowledge concerning diagnosis, pathogenesis and treatment of the cardiac and pulmonary lupus manifestations are discussed.
Review of the literature.
Although pericarditis is the most frequent heart manifestation in the context of lupus, valvular disease and less often myocarditis may be detected. In the latter, treatment should be prompt and aggressive to prevent chronic sequelae like congestive heart failure. Later on disease course, accelerated atherosclerosis is considered as one of the most important co-morbidities of SLE with cardiovascular events being one of the leading causes of death at relatively young ages. Stratification of the patients at risk and stringent management of the traditional risk factors are warranted. Respiratory system involvement affects all anatomic structures of the lungs, pleura and pulmonary vasculature while its severity ranges from asymptomatic pleural disease to acute respiratory failure. The most common features include pleuritis, interstitial lung disease and pulmonary embolism on the background of antiphospholipid syndrome. Less usual complications include lupus pneumonitis, diffuse alveolar hemorrhage, shrinking lung syndrome and pulmonary arterial hypertension.
There are no specific guidelines for the management of these manifestations and therapeutic approach remains empiric.
系统性红斑狼疮(SLE)具有多种临床表现和免疫异常特征。心血管和呼吸系统受累对患者预后的重要性日益受到认可。在本综述中,讨论了有关心脏和肺部狼疮表现的诊断、发病机制及治疗的现有知识。
文献综述。
虽然心包炎是狼疮患者最常见的心脏表现,但也可能检测到瓣膜病,心肌炎则较少见。对于心肌炎,应迅速积极治疗以预防诸如充血性心力衰竭等慢性后遗症。在疾病后期,加速动脉粥样硬化被认为是SLE最重要的合并症之一,心血管事件是相对年轻患者死亡的主要原因之一。有必要对高危患者进行分层并严格管理传统危险因素。呼吸系统受累影响肺、胸膜和肺血管的所有解剖结构,其严重程度从无症状胸膜疾病到急性呼吸衰竭不等。最常见的特征包括胸膜炎、间质性肺疾病以及抗磷脂综合征背景下的肺栓塞。较少见的并发症包括狼疮肺炎、弥漫性肺泡出血、肺萎陷综合征和肺动脉高压。
对于这些表现的管理尚无具体指南,治疗方法仍基于经验。