成人发作的斯蒂尔病(AOSD)是一种多基因的全身性自身炎症性疾病,与发病率和死亡率增加有关。肺部受累是罕见的,而是AOSD的严重并发症。在AOSD中,IL-1b,IL-18和IL-6在分子发病机制中占主导地位,介导适应性免疫系统的1型和3型炎症特征。IL-1-和IL-6抑制在AOSD管理中的成功证明了这一点。然而,目前正在讨论对IL-1-或IL-6抑制剂治疗的过敏反应是AOSD肺部受累的潜在触发因素,遗传风险因素也已被确定。临床上,AOSD的肺部受累可以表现为许多不同的形式。伴有外周巩固的实质炎症是最常见的形式,而PAH则较少见。但往往很难管理。这篇小型综述概述了AOSD中肺部受累的病理生理学以及临床表现和诊断特征。
Adult-onset Still\'s disease (AOSD) is a polygenic systemic autoinflammatory disease which is associated with increased morbidity and mortality. Pulmonary involvement is a rare, but serious complication of AOSD. As in AOSD, IL-1b, IL-18, and IL-6 dominate the molecular pathogenesis, which mediate a type 1 and type 3 inflammatory signature of the adaptive immune system. This is evidenced by the success of IL-1- and IL-6 inhibition in the management of AOSD. However, anaphylactic reactions to treatment with IL-1- or IL-6-inhibitors is currently being discussed as a potential trigger for lung involvement inf AOSD, while genetic risk factors have also been identified. Clinically, pulmonary involvement in AOSD can manifest in many different forms. Parenchymal inflammation with peripheral consolidations is the most frequent form while PAH is less common, but often very difficult to manage. This mini-review provides an overview of the pathophysiology as well as the clinical presentation and the diagnostic features of pulmonary involvement in AOSD.