Type 2 innate lymphoid cell counts are increased in patients with systemic sclerosis and correlate with the extent of fibrosis
Type 2 innate lymphoid cell counts are increased in patients with systemic sclerosis and correlate with the extent of fibrosis
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DOI：
10.1136/annrheumdis-2015-207388
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发表时间：
2016-03-01
影响因子：
27.4
通讯作者：
Ramming, Andreas
中科院分区：
文献类型：
--
作者：
Wohlfahrt, Thomas;Usherenko, Svetlana;Ramming, Andreas
目的2型先天性淋巴样细胞（Type 2 innate lymphoid cells，ILC2）是近年发现的一类缺乏谱系特异性受体的淋巴样细胞，具有促进2型免疫和组织重建的功能。然而，ILC2在系统性硬化症（SSc）发病机制中的作用尚不清楚。我们的目的是评估的水平和相关性与SSc的纤维化表现。方法69例SSc患者和47名健康对照。血液样本和皮肤切片进行了分析，流式细胞术和免疫化学染色两个互补的面板markers.Results皮肤和循环ILC2与SSc患者与对照组相比，显着升高。皮肤，但不是循环ILC2被激活。对局限性皮肤SSc（lcSSc）和弥漫性皮肤SSc（dcSSc）患者的SSc人群进行分层，结果表明两个亚组中ILC2水平均升高，dcSSc的频率显著高于lcSSc。此外，皮肤和循环ILC2计数密切相关的修改Rodnan皮肤评分和存在的肺fibrosis.Conclusions ILC2计数升高与SSc患者的皮肤纤维化的程度和间质性肺病的存在提供令人信服的证据ILC2在SSc的促纤维化作用。
Objective Type 2 innate lymphoid cells (ILC2s), a recently identified population of lymphoid cells lacking lineage-specific receptors, promote type 2 immunity and tissue remodelling. However, the contributive role of ILC2s in the pathogenesis of systemic sclerosis (SSc) is unknown. We aimed to evaluate the levels and correlations with fibrotic manifestations in SSc.Methods 69 patients with SSc and 47 healthy controls were included. Blood samples and skin sections were analysed by flow cytometry and immunohistochemically by staining two complementary panels of markers.Results Dermal and circulating ILC2s were significantly elevated in patients with SSc compared with controls. Dermal, but not circulating ILC2s were activated. Stratification of the SSc population in patients with limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) demonstrated increased levels of ILC2s in both subgroups with significantly higher frequencies in dcSSc compared with lcSSc. Moreover, dermal and circulating ILC2 counts correlated closely with the modified Rodnan skin score and with the presence of pulmonary fibrosis.Conclusions ILC2 counts are elevated in patients with SSc and correlate with the extent of skin fibrosis and the presence of interstitial lung disease providing compelling evidence for profibrotic effect of ILC2s in SSc.