Dendritic Cells Are the Intriguing Players in the Puzzle of Idiopathic Pulmonary Fibrosis Pathogenesis.
Dendritic Cells Are the Intriguing Players in the Puzzle of Idiopathic Pulmonary Fibrosis Pathogenesis.
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DOI：
10.3389/fimmu.2021.664109
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发表时间：
2021
影响因子：
7.3
通讯作者：
Galati D
中科院分区：
文献类型：
--
作者：
Bocchino M;Zanotta S;Capitelli L;Galati D
特发性肺纤维化（IPF）是最具破坏性的进行性间质性肺病，仍然难以治疗。IPF的发病机制依赖于受损肺泡细胞和肌成纤维细胞之间的异常串扰，最终导致异常纤维反应。免疫系统对IPF的作用仍未得到充分研究。最近的证据表明，先天性和适应性免疫反应都可能参与纤维化过程。树突状细胞（Dendritic cells，DCs）是最强的专职抗原提呈细胞，在先天免疫和获得性免疫之间起着桥梁作用。此外，它们在肺的免疫监视中发挥关键作用，其中它们被战略性地放置在气道上皮和肺泡中。未成熟DC在靠近上皮增生和纤维化区域的IPF肺中积聚。相反，成熟DC集中在组织良好的淋巴滤泡中，沿着IPF患者的T和B细胞和支气管肺泡灌洗液中。我们最近发现，在未经治疗的IPF患者中，所有外周血DC亚型（包括常规和浆细胞样DC）均严重耗竭。此外，常规CD1c+ DC的低频率预示着更差的预后。本小型综述的目的是关注DC参与IPF发病机制的主要证据。未回答的问题和未来研究的机会，从更好地了解他们的诊断和预后的贡献，以个性化的DC为基础的治疗将进行探讨。
Idiopathic pulmonary fibrosis (IPF) is the most devastating progressive interstitial lung disease that remains refractory to treatment. Pathogenesis of IPF relies on the aberrant cross-talk between injured alveolar cells and myofibroblasts, which ultimately leads to an aberrant fibrous reaction. The contribution of the immune system to IPF remains not fully explored. Recent evidence suggests that both innate and adaptive immune responses may participate in the fibrotic process. Dendritic cells (DCs) are the most potent professional antigen-presenting cells that bridge innate and adaptive immunity. Also, they exert a crucial role in the immune surveillance of the lung, where they are strategically placed in the airway epithelium and interstitium. Immature DCs accumulate in the IPF lung close to areas of epithelial hyperplasia and fibrosis. Conversely, mature DCs are concentrated in well-organized lymphoid follicles along with T and B cells and bronchoalveolar lavage of IPF patients. We have recently shown that all sub-types of peripheral blood DCs (including conventional and plasmacytoid DCs) are severely depleted in therapy naïve IPF patients. Also, the low frequency of conventional CD1c+ DCs is predictive of a worse prognosis. The purpose of this mini-review is to focus on the main evidence on DC involvement in IPF pathogenesis. Unanswered questions and opportunities for future research ranging from a better understanding of their contribution to diagnosis and prognosis to personalized DC-based therapies will be explored.
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影响因子：
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作者：
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通讯作者：
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DOI：
10.1084/jem.20131397
发表时间：
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期刊：
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