Valeur Natalie S, Stevens Anne M, Ferguson Mark R, Effmann Eric L, Iyer Ramesh S
1 Department of Radiology, University of Washington Medical Center, Seattle, WA.
AJR Am J Roentgenol. 2015 Feb;204(2):408-22. doi: 10.2214/AJR.14.12461.
OBJECTIVE. Juvenile systemic sclerosis is a rare multisystem autoimmune disorder characterized by vasculopathy and multiorgan fibrosis. Cardiopulmonary complications are the leading cause of morbidity and mortality. Although pulmonary fibrosis is the complication that is most common and well described, cardiovascular and esophageal involvement may also be observed. In this article, common thoracic findings in juvenile systemic sclerosis will be discussed. We will focus on chest CT, including CT findings of pulmonary fibrosis and associated grading methods, as well as cardiac MRI and esophageal imaging. CONCLUSION. Radiologists play a pivotal role in the initial diagnosis and follow-up evaluation of pediatric patients with systemic sclerosis. Treatment decisions and prognostic assessment are directly related to imaging findings along with clinical evaluation.
目的。青少年系统性硬化症是一种罕见的多系统自身免疫性疾病，其特征为血管病变和多器官纤维化。心肺并发症是发病和死亡的主要原因。虽然肺纤维化是最常见且描述详尽的并发症，但也可能观察到心血管和食管受累情况。在本文中，将讨论青少年系统性硬化症常见的胸部表现。我们将重点关注胸部CT，包括肺纤维化的CT表现及相关分级方法，以及心脏MRI和食管成像。结论。放射科医生在系统性硬化症儿科患者的初始诊断和随访评估中发挥着关键作用。治疗决策和预后评估与影像学表现以及临床评估直接相关。
