Immunopathogenesis Section, Program in Barrier Immunity and Repair, Laboratory of Parasitic Diseases, National Institute of Allergy and Infectious Diseases, National Institutes of Health, Bethesda, Maryland, USA.
Nat Med. 2012 Jul 6;18(7):1028-40. doi: 10.1038/nm.2807.
Fibrosis is a pathological feature of most chronic inflammatory diseases. Fibrosis, or scarring, is defined by the accumulation of excess extracellular matrix components. If highly progressive, the fibrotic process eventually leads to organ malfunction and death. Fibrosis affects nearly every tissue in the body. Here we discuss how key components of the innate and adaptive immune response contribute to the pathogenesis of fibrosis. We also describe how cell-intrinsic changes in important structural cells can perpetuate the fibrotic response by regulating the differentiation, recruitment, proliferation and activation of extracellular matrix-producing myofibroblasts. Finally, we highlight some of the key mechanisms and pathways of fibrosis that are being targeted as potential therapies for a variety of important human diseases.
纤维化是大多数慢性炎症性疾病的病理特征。纤维化或瘢痕形成定义为细胞外基质成分的过度积累。如果进展迅速，纤维化过程最终会导致器官功能障碍和死亡。纤维化影响身体的几乎每一种组织。在这里，我们讨论先天和适应性免疫反应的关键成分如何导致纤维化的发病机制。我们还描述了重要结构细胞中细胞内变化如何通过调节细胞外基质产生的肌成纤维细胞的分化、募集、增殖和激活来维持纤维化反应。最后，我们强调了纤维化的一些关键机制和途径，这些机制和途径正被作为各种重要人类疾病的潜在治疗方法进行研究。
