It is thought that systemic sclerosis (SSc) might be a T helper 17 (Th17) cell-driven autoimmune disease. Noticeably, pulmonary arterial hypertension (PAH) is a leading cause of death in patients with SSc. Here, we investigated the association between serum Th17-related cytokines and prevalence of PAH in SSc patients. This study included 72 SSc patients and 51 healthy controls (HC). We determined clinical manifestations, immunophenotypes including Th subsets in peripheral blood lymphocytes, and the serum levels of interleukin (IL)-17A, IL-17A/F, IL-17B. IL-17C, IL-17D. IL-1β, IL-6, IL-21, IL-22, and IL-23. The frequency of Th17 cells was significantly increased in SSc patients compared to HC and was positively correlated with the modified Rodnan skin scores. Furthermore, the serum levels of IL-17A, IL-17D, IL-1β, and IL-6 were significantly increased in SSc patients compared to HC. SSc patients with detected IL-17A showed high levels of IL-17A/F, IL-1β, IL-6, and IL-22, and high frequency of Th17 cells. Interestingly, these patients exhibited the reduced lung functions and increased prevalence of PAH significantly compared to patients with undetected IL-17A. Similarly, SSc patients with detected IL-17A and high IL-6 (≥1.2 pg/mL) exhibited the decreased lung functions and increased prevalence of PAH compared to patients with undetected IL-17A and low IL-6. We found that SSc patients with high levels of serum IL-17A or both IL-17A and IL-6 show reduced lung functions and high prevalence of PAH. Consequently, it is highly probable that Th17/IL-17A axis is critical for the prevalence of PAH in SSc patients.

中文翻译：

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Th17/IL-17A 轴对于系统性硬化症 (SSc) 中的肺动脉高压 (PAH) 至关重要：血清 IL-17A 水平高的 SSc 患者表现出肺功能下降和 PAH 患病率增加

人们认为系统性硬化症 (SSc) 可能是一种辅助性 T 细胞 17 (Th17) 细胞驱动的自身免疫性疾病。值得注意的是，肺动脉高压（PAH）是 SSc 患者死亡的主要原因。在这里，我们研究了 SSc 患者血清 Th17 相关细胞因子与 PAH 患病率之间的关联。这项研究包括 72 名 SSc 患者和 51 名健康对照 (HC)。我们测定了临床表现、免疫表型（包括外周血淋巴细胞中的 Th 亚群）以及白细胞介素 (IL)-17A、IL-17A/F、IL-17B 的血清水平。IL-17C、IL-17D。IL-1β、IL-6、IL-21、IL-22 和 IL-23。与 HC 患者相比，SSc 患者的 Th17 细胞频率显着增加，并且与改良的 Rodnan 皮肤评分呈正相关。此外，与 HC 患者相比，SSc 患者的血清 IL-17A、IL-17D、IL-1β 和 IL-6 水平显着升高。检测到 IL-17A 的 SSc 患者表现出高水平的 IL-17A/F、IL-1β、IL-6 和 IL-22，以及高频率的 Th17 细胞。有趣的是，与未检测到 IL-17A 的患者相比，这些患者的肺功能明显下降，PAH 患病率显着增加。同样，与未检测到 IL-17A 和低 IL-6 的患者相比，检测到 IL-17A 和高 IL-6 (≥1.2 pg/mL) 的 SSc 患者表现出肺功能下降和 PAH 患病率增加。我们发现，血清 IL-17A 或 IL-17A 和 IL-6 水平较高的 SSc 患者肺功能下降，PAH 患病率较高。因此，Th17/IL-17A 轴很可能对于 SSc 患者 PAH 的患病率至关重要。