Medical Intensive Care Unit, Service de Médecine Intensive-Réanimation, CHU Grenoble-Alpes, Université Grenoble-Alpes, INSERM, U1042-HP2, Grenoble, France.
Intensive Care Department, Ministry of the National Guard-Health Affairs, Riyadh, Kingdom of Saudi Arabia.
Intensive Care Med. 2024 Jan;50(1):17-35. doi: 10.1007/s00134-023-07266-7. Epub 2023 Dec 19.
Autoimmune diseases encompass a broad spectrum of disorders characterized by disturbed immunoregulation leading to the development of specific autoantibodies, resulting in inflammation and multiple organ involvement. A distinction should be made between connective tissue diseases (mainly systemic lupus erythematosus, systemic scleroderma, inflammatory muscle diseases, and rheumatoid arthritis) and vasculitides (mainly small-vessel vasculitis such as antineutrophil cytoplasmic antibody-associated vasculitis and immune-complex mediated vasculitis). Admission of patients with autoimmune diseases to the intensive care unit (ICU) is often triggered by disease flare-ups, infections, and organ failure and is associated with high mortality rates. Management of these patients is complex, including prompt disease identification, immunosuppressive treatment initiation, and life-sustaining therapies, and requires multi-disciplinary involvement. Data about autoimmune diseases in the ICU are limited and there is a need for multicenter, international collaboration to improve patients' diagnosis, management, and outcomes. The objective of this narrative review is to summarize the epidemiology, clinical features, and selected management of severe systemic autoimmune diseases.
自身免疫性疾病包括一大类疾病，其特征是免疫调节紊乱导致特定自身抗体的产生，从而引起炎症和多器官受累。应区分结缔组织疾病（主要是系统性红斑狼疮、系统性硬皮病、炎症性肌病和类风湿关节炎）和血管炎（主要是小血管血管炎，如抗中性粒细胞胞质抗体相关性血管炎和免疫复合物介导的血管炎）。自身免疫性疾病患者入住重症监护病房（ICU）通常是由疾病发作、感染和器官衰竭引起的，并且与高死亡率相关。这些患者的管理很复杂，包括及时识别疾病、开始免疫抑制治疗和维持生命的治疗，需要多学科参与。关于 ICU 中自身免疫性疾病的数据有限，需要多中心、国际合作来改善患者的诊断、管理和预后。本叙述性综述的目的是总结严重系统性自身免疫性疾病的流行病学、临床特征和特定治疗方法。
