2 型固有淋巴细胞计数在系统性硬化症患者中增加，并与纤维化程度相关。

Type 2 innate lymphoid cell counts are increased in patients with systemic sclerosis and correlate with the extent of fibrosis.

机构信息

Department of Internal Medicine 3, Institute for Clinical Immunology, University of Erlangen-Nuremberg, Erlangen, Germany.

Department of Trauma Surgery, University of Erlangen-Nuremberg, Erlangen, Germany.

出版信息

Ann Rheum Dis. 2016 Mar;75(3):623-6. doi: 10.1136/annrheumdis-2015-207388. Epub 2015 Sep 2.

DOI:10.1136/annrheumdis-2015-207388

PMID:26338035

Abstract

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.

摘要

目的

2 型先天淋巴样细胞（ILC2s）是一种最近被鉴定出的缺乏谱系特异性受体的淋巴样细胞群体，它促进 2 型免疫和组织重塑。然而，ILC2s 在系统性硬化症（SSc）发病机制中的作用尚不清楚。我们旨在评估其在 SSc 中的水平及其与纤维化表现的相关性。

方法

纳入 69 例 SSc 患者和 47 例健康对照者。通过流式细胞术和免疫组织化学染色两种互补的标记物分析血液样本和皮肤切片。

结果

与对照组相比，SSc 患者真皮和循环 ILC2s 显著升高。真皮，而不是循环 ILC2s 被激活。对局限性皮肤 SSc（lcSSc）和弥漫性皮肤 SSc（dcSSc）的 SSc 人群进行分层，显示两组 ILC2s 水平均升高，dcSSc 明显高于 lcSSc。此外，真皮和循环 ILC2 计数与改良 Rodnan 皮肤评分和肺纤维化的存在密切相关。