Kolahian Saeed, Fernandez Isis E, Eickelberg Oliver, Hartl Dominik
1 Pediatric Infectiology, Immunology, and Cystic Fibrosis, Children's Hospital of the University of Tübingen, Tübingen, Germany.
2 Comprehensive Pneumology Center, Institute of Lung Biology and Disease, University Hospital, Ludwig Maximilians University and Helmholtz Zentrum München, Member of the German Center for Lung Research, Munich, Germany; and.
Am J Respir Cell Mol Biol. 2016 Sep;55(3):309-22. doi: 10.1165/rcmb.2016-0121TR.
Pulmonary fibrosis, particularly idiopathic pulmonary fibrosis, represents a chronic and progressive disease with high mortality and limited therapeutic options. Excessive deposition of extracellular matrix proteins results in fibrotic remodeling, alveolar destruction, and irreversible loss of lung function. Both innate and adaptive immune mechanisms contribute to fibrogenesis at several cellular and noncellular levels. Here, we summarize and discuss the role of immune cells (T cells, neutrophils, macrophages, and fibrocytes) and soluble mediators (cytokines and chemokines) involved in pulmonary fibrosis, pointing toward novel immune-based therapeutic strategies in the field.
肺纤维化,尤其是特发性肺纤维化,是一种慢性进行性疾病,死亡率高且治疗选择有限。细胞外基质蛋白的过度沉积导致纤维化重塑、肺泡破坏和肺功能的不可逆丧失。先天性和适应性免疫机制在多个细胞和非细胞水平上都对纤维化的形成有作用。在此,我们总结并讨论了参与肺纤维化的免疫细胞(T细胞、中性粒细胞、巨噬细胞和纤维细胞)和可溶性介质(细胞因子和趋化因子)的作用,为该领域基于免疫的新型治疗策略指明方向。