Background. Few biomarkers are available for early identification of pulmonary arterial hypertension (PAH) and interstitial lung disease (ILD) in systemic sclerosis (SS) and scleroderma spectrum disorders (SSD). Aims. To evaluate Gas6, sAxl, and sMer as biomarkers for cardiopulmonary complications of SS and SSD. Methods. In a cross-sectional observational study, we recruited 125 consecutive patients, affected by SS and SSD and referred to a tertiary-level pulmonary hypertension outpatient clinic. All patients underwent a comprehensive evaluation for identification of PAH and ILD. Gas6, sMer, and sAxl concentrations were measured with ELISA protocols, and concentrations were compared according to PAH or ILD. Results. Nineteen subjects had pulmonary hypertension (PH) (14 PAH), and 39 had ILD (6 severe). Plasma sMer was increased in PAH (18.6 ng/ml IQR [11.7-20.3]) with respect to the absence (12.4 [8.0-15.8]) or other form of pulmonary hypertension (9.6 [7.4-12.5]; K–W variance ). Conversely, Gas6 and sAxl levels were slightly increased in mild ILD (25.8 ng/ml [19.5-32.1] and 24.6 [20.1-32.5]) and reduced in severe ILD (16.6 [15.0-22.1] and 15.5 [14.9-22.4]) in comparison to no evidence of ILD (23.4 [18.8-28.1] and 21.6 [18.1-28.4]; K–W, ). Plasma has 50% sensitivity and 92% specificity in PAH identification (area under the ROC curve (AUC) 0.697, ). Values of and of have 100% and 67% sensitivity and 47% and 86% specificity, respectively, in identifying severe ILD (Gas6 AUC 0.787, ; sAxl AUC 0.705, ). Conclusions. The assay of Gas6 sAxl and sMer may be useful to help in the identification of PAH and ILD in SS and SSD patients. The Gas6/TAM system seems to be relevant in cardiopulmonary complications of SS and SSD and merits further investigations.

中文翻译：

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Gas6 和 TAM 受体在识别系统性硬化症和硬皮病谱系疾病心肺参与中的作用。

背景。很少有生物标志物可用于早期识别系统性硬化症 (SS) 和硬皮病谱系疾病 (SSD) 中的肺动脉高压 (PAH) 和间质性肺病 (ILD)。目标。评估 Gas6、sAxl 和 sMer 作为 SS 和 SSD 心肺并发症的生物标志物。方法。在一项横断面观察研究中，我们连续招募了 125 名受 SS 和 SSD 影响的患者，并转诊至三级肺动脉高压门诊。所有患者都接受了综合评估，以确定 PAH 和 ILD。使用 ELISA 协议测量 Gas6、sMer 和 sAxl 浓度，并根据 PAH 或 ILD 比较浓度。结果. 19 名受试者患有肺动脉高压 (PH) (14 PAH)，39 名患有 ILD（6 名严重）。血浆 sMer 在 PAH (18.6 ng/ml IQR [11.7-20.3]) 中相对于缺乏 (12.4 [8.0-15.8]) 或其他形式的肺动脉高压 (9.6 [7.4-12.5]; K-W 方差）。相反，Gas6 和 sAxl 水平在轻度 ILD 中略有增加（25.8 ng/ml [19.5-32.1] 和 24.6 [20.1-32.5]），在重度 ILD 中降低（16.6 [15.0-22.1] 和 15.5 [14.9-22.4]）与无 ILD 证据相比（23.4 [18.8-28.1] 和 21.6 [18.1-28.4]；K-W，）。等离子体在 PAH 鉴定中具有 50% 的敏感性和 92% 的特异性（ROC 曲线下面积 (AUC) 0.697，）。的价值观和在识别重度 ILD 方面分别具有 100% 和 67% 的敏感性和 47% 和 86% 的特异性（Gas6 AUC 0.787，; sAxl AUC 0.705,）。 结论。Gas6 sAxl 和 sMer 的测定可能有助于识别 SS 和 SSD 患者的 PAH 和 ILD。Gas6/TAM 系统似乎与 SS 和 SSD 的心肺并发症有关，值得进一步研究。