Hoyne Gerard F, Elliott Hannah, Mutsaers Steven E, Prêle Cecilia M
School of Health Sciences, University of Notre Dame Australia, Fremantle, Western Australia, Australia.
Institute of Health Research, University of Notre Dame Australia, Fremantle, Western Australia, Australia.
Immunol Cell Biol. 2017 Aug;95(7):577-583. doi: 10.1038/icb.2017.22. Epub 2017 Mar 30.
Idiopathic pulmonary fibrosis (IPF) is the most common of the idiopathic interstitial pneumonias. It is typically associated with extensive and progressive fibrosis, and is fatal and has limited treatment options. Characteristically IPF patients display large lymphocyte aggregates composed of CD3 T cells and CD20 B cells within the lung tissue that are located near sites of active fibrosis. In addition, IPF patients can have autoantibodies to a range of host antigens, suggesting a breakdown in immunological tolerance. In this review, we examine the role of T and B cells in IPF pathogenesis and discuss how loss of self-tolerance to lung-specific proteins could exacerbate disease progression in IPF. We discuss what these results mean in terms of future prospects for immunotherapy of IPF.
特发性肺纤维化（IPF）是最常见的特发性间质性肺炎。它通常与广泛且进行性的纤维化相关，是致命性疾病且治疗选择有限。IPF患者的特征是肺组织内存在由CD3 T细胞和CD20 B细胞组成的大淋巴细胞聚集物，这些聚集物位于活跃纤维化部位附近。此外，IPF患者可产生针对一系列宿主抗原的自身抗体，提示免疫耐受出现破坏。在本综述中，我们研究了T细胞和B细胞在IPF发病机制中的作用，并讨论了对肺特异性蛋白的自身耐受丧失如何可能加剧IPF的疾病进展。我们还讨论了这些结果对IPF免疫治疗未来前景的意义。
