Department of Internal Medicine, Gazi University Faculty of Medicine, Ankara, Turkey.
Department of Internal Medicine, Division of Geriatrics, Istanbul University-Cerrahpasa, Cerrahpasa Faculty of Medicine, Istanbul, Turkey.
Adv Rheumatol. 2020 Dec 22;60(1):54. doi: 10.1186/s42358-020-00156-2.
Systemic sclerosis (SSc) is an autoimmune disease characterized by fibrosis of skin and lung as well as involvement of kidney, gastrointestinal system and heart. Aetiology and exact mechanism of disease is poorly understood. The association between antimicrobial peptides (AMPs) and other diseases such as idiopathic pulmonary fibrosis, diffuse panbronchiolitis, pulmoner alveolar proteinosis and psoriasis have been reported. A small number of studies have examined the role of AMPs on autoimmune diseases which has not been studied in scleroderma yet. We aimed to investigate AMP serum levels and their association with disease characteristics of SSc.
Forty-two patients (40 female, mean age 42 years) and 38 healthy subjects (32 female, mean age 38 years) were enrolled. For SSc patients, the following data were recorded: disease subset (limited/diffuse), autoantibodies (antinuclear, anti-centromere (ACA), and anti-SCL-70), blood tests, erythrocyte sedimentation rate (ESR), and C-reactive protein (CRP), modified Rodnan skin score, presence and history of digital ulcers, kidney, gastrointestinal disease and lung involvement assessed by computed tomography and pulmonary function tests. Association between serum AMPs and disease characteristics were analysed.
Twenty-nine of the patients had diffuse (69%) and 13 of the patients had limited (31%) systemic sclerosis. Average disease duration was 5.5 years. Pulmonary involvement was detected in 20 patients (47.6%). Serum concentration of alpha defensin was higher than healthy subjects (563 ± 415 vs 377 ± 269 ng/mL, p = 0.02). However, no difference was observed for beta-1 and beta-2 defensins in SSc patients and healthy controls. In sub-group analysis patients with interstitial lung disease had higher levels of alpha defensin than those without lung involvement (684 ± 473 vs 430 ± 299 ng/ml, p = 0.04). There was also correlation between alfa defensin serum concentrations and CRP (r = 0.34).
Alpha defensin levels are increased in scleroderma patients and correlated with lung involvement indicating a role in the pathogenesis of disease.
This study is not a clinical trial study.
系统性硬化症（SSc）是一种自身免疫性疾病，其特征为皮肤和肺部纤维化，以及肾脏、胃肠道系统和心脏受累。其病因和确切发病机制尚不清楚。已报道抗菌肽（AMPs）与特发性肺纤维化、弥漫性泛细支气管炎、肺泡蛋白沉积症和银屑病等其他疾病之间存在关联。少数研究检查了 AMPs 对自身免疫性疾病的作用，但尚未在硬皮病中进行研究。我们旨在研究 AMP 血清水平及其与 SSc 疾病特征的关系。
共纳入 42 名患者（40 名女性，平均年龄 42 岁）和 38 名健康对照者（32 名女性，平均年龄 38 岁）。对 SSc 患者记录以下数据：疾病亚型（局限性/弥漫性）、自身抗体（抗核、抗着丝点（ACA）和抗 SCL-70）、血液检查、红细胞沉降率（ESR）和 C 反应蛋白（CRP）、改良 Rodnan 皮肤评分、有无并病史的手指溃疡、肾脏、胃肠道疾病和通过计算机断层扫描和肺功能检查评估的肺部受累。分析血清 AMPs 与疾病特征之间的关系。
29 名患者为弥漫性（69%），13 名患者为局限性（31%）系统性硬化症。平均病程为 5.5 年。20 名患者（47.6%）有肺部受累。α防御素的血清浓度高于健康对照组（563±415 vs 377±269ng/ml，p=0.02）。然而，SSc 患者和健康对照组的β-1 和β-2 防御素无差异。亚组分析显示，间质性肺病患者的α防御素水平高于无肺部受累者（684±473 vs 430±299ng/ml，p=0.04）。α防御素血清浓度与 CRP 之间也存在相关性（r=0.34）。
α防御素水平在硬皮病患者中升高，并与肺部受累相关，提示其在疾病发病机制中起作用。
