Nies Jasper F, Schneider Udo, Krusche Martin
Internal Medicine II, University Medical Center Cologne, Cologne, Germany.
Department of Rheumatology and Clinical Immunology, Charité - Universitätsmedizin Berlin, Berlin, Germany.
Front Med (Lausanne). 2022 Sep 16;9:989777. doi: 10.3389/fmed.2022.989777. eCollection 2022.
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.
成人斯蒂尔病（AOSD）是一种多基因系统性自身炎症性疾病，与发病率和死亡率增加相关。肺部受累是AOSD罕见但严重的并发症。与AOSD一样，白细胞介素-1β（IL-1β）、白细胞介素-18（IL-18）和白细胞介素-6（IL-6）在分子发病机制中起主导作用，它们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治疗中的成功证明了这一点。然而，目前正在讨论IL-1或IL-6抑制剂治疗引起的过敏反应是AOSD肺部受累的潜在触发因素，同时也已确定了遗传风险因素。临床上，AOSD的肺部受累可表现为多种不同形式。伴有外周实变的实质炎症是最常见的形式，而肺动脉高压（PAH）较少见，但通常很难处理。本综述概述了AOSD肺部受累的病理生理学、临床表现和诊断特征。
