Graduate Institute of Clinical Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan.
School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung 807, Taiwan.
Int J Mol Sci. 2023 Jul 26;24(15):11995. doi: 10.3390/ijms241511995.
Connective tissue disease-associated interstitial lung disease (CTD-ILD) is a severe manifestation of CTD that leads to significant morbidity and mortality. Clinically, ILD can occur in diverse CTDs. Pathologically, CTD-ILD is characterized by various histologic patterns, such as nonspecific interstitial pneumonia, organizing pneumonia, and usual interstitial pneumonia. Abnormal immune system responses have traditionally been instrumental in its pathophysiology, and various changes in immune cells have been described, especially in macrophages. This article first briefly overviews the epidemiology, clinical characteristics, impacts, and histopathologic changes associated with CTD-ILD. Next, it summarizes the roles of various signaling pathways in macrophages or products of macrophages in ILD, helped by insights gained from animal models. In the following sections, this review returns to studies of macrophages in CTD-ILD in humans for an overall picture of the current understanding. Finally, we direct attention to potential therapies targeting macrophages in CTD-ILD in investigation or in clinical trials, as well as the future directions regarding macrophages in the context of CTD-ILD. Although the field of macrophages in CTD-ILD is still in its infancy, several lines of evidence suggest the potential of this area.
结缔组织病相关性间质性肺疾病（CTD-ILD）是 CTD 的严重表现形式，可导致显著的发病率和死亡率。临床上，ILD 可发生于多种 CTD。从病理学角度来看，CTD-ILD 的特征是存在各种组织学模式，如非特异性间质性肺炎、机化性肺炎和寻常间质性肺炎。异常的免疫系统反应一直是其病理生理学的关键因素，已经描述了各种免疫细胞的变化，尤其是巨噬细胞。本文首先简要概述了 CTD-ILD 的流行病学、临床特征、影响因素和组织病理学变化。接下来，它通过动物模型获得的见解，总结了各种信号通路在巨噬细胞或巨噬细胞产物在 ILD 中的作用。在接下来的部分中，本综述回顾了人类 CTD-ILD 中巨噬细胞的研究，以全面了解目前的认识。最后，我们将注意力转向针对 CTD-ILD 中巨噬细胞的潜在治疗方法，包括正在调查或临床试验中的治疗方法，以及 CTD-ILD 背景下巨噬细胞的未来方向。尽管 CTD-ILD 中巨噬细胞的研究领域仍处于起步阶段，但有几项证据表明该领域具有潜力。
