Hizal Mutlu, Bruni Cosimo, Romano Eloisa, Mazzotta Celestina, Guiducci Serena, Bellando Randone Silvia, Blagojevic Jelena, Lepri Gemma, Tufan Abdurrahman, Matucci Cerinic Marco
Internal Medicine Department, Gazi University Medical Faculty, Ankara, Turkey,
Clin Rheumatol. 2015 Apr;34(4):795-8. doi: 10.1007/s10067-014-2854-1. Epub 2015 Jan 20.
Interstitial lung disease (ILD) is the leading cause of systemic sclerosis (SSc) related morbidity and mortality. LL-37 peptide is the only cathelicidin of the human antimicrobial peptide family with antimicrobial effects and immunomodulatory activity. LL-37 has anti-fibrotic effects and anti-apoptotic effects on SSc dermal fibroblasts. The aim of the study was to investigate the circulating levels of LL-37 in SSc patients and its association with clinical, laboratory, and instrumental parameters. Fifty-eight SSc patients (30 with and 28 without pulmonary involvement) and 28 healthy controls were enrolled in the study. Pulmonary involvement was defined when ILD was found at HRCT (ground glass, reticular, and honeycombing pattern). Circulating LL-37 levels were measured with ELISA test. In SSc patients with ILD serum, LL-37 concentrations were remarkably lower (1.36 mg/ml) than those in SSc patients without ILD (4.62 ng/ml, p = 0.035) and controls (5.53 ng/ml, p = 0.009). In SSc patients without ILD, serum LL-37 levels were not different from controls (p = 0.812). No significant association or correlation was found between LL-37 levels and any other clinical, serological, or instrumental features. Serum LL-37 levels are significantly lower in patients with SSc ILD. Our results may suggest that lower LL-37 levels may be associated with the development of ILD. Whether circulating levels of LL-37 might be used as an indirect marker of ILD remains to be determined in larger SSc cohorts.
间质性肺疾病（ILD）是系统性硬化症（SSc）相关发病和死亡的主要原因。LL-37肽是人类抗菌肽家族中唯一具有抗菌作用和免疫调节活性的cathelicidin。LL-37对SSc皮肤成纤维细胞具有抗纤维化作用和抗凋亡作用。本研究的目的是调查SSc患者中LL-37的循环水平及其与临床、实验室和仪器参数的关联。58例SSc患者（30例有肺部受累，28例无肺部受累）和28例健康对照者纳入本研究。当在高分辨率计算机断层扫描（HRCT）上发现ILD（磨玻璃、网状和蜂窝状模式）时定义为肺部受累。用酶联免疫吸附测定（ELISA）试验测量循环LL-37水平。在有ILD血清的SSc患者中，LL-37浓度（1.36毫克/毫升）显著低于无ILD的SSc患者（4.62纳克/毫升，p = 0.035）和对照组（5.53纳克/毫升，p = 0.009）。在无ILD的SSc患者中，血清LL-37水平与对照组无差异（p = 0.812）。未发现LL-37水平与任何其他临床、血清学或仪器特征之间存在显著关联或相关性。SSc-ILD患者的血清LL-37水平显著降低。我们的结果可能表明较低的LL-37水平可能与ILD的发生有关。LL-37的循环水平是否可作为ILD的间接标志物仍有待在更大的SSc队列中确定。
