Pawlik Katarzyna Karina, Bohdziewicz Anna, Chrabąszcz Magdalena, Stochmal Anna, Sikora Mariusz, Alda-Malicka Rosanna, Czuwara Joanna, Rudnicka Lidia
STUDENT RESEARCH COMMITTEE, DEPARTMENT OF DERMATOLOGY, MEDICAL UNIVERSITY OF WARSAW, WARSAW, POLAND.
DEPARTMENT OF DERMATOLOGY, MEDICAL UNIVERSITY OF WARSAW, WARSAW, POLAND.
Wiad Lek. 2020;73(10):2300-2305.
Systemic sclerosis (SSc) is an autoimmune disease characterized by vasculopathy and uncontrolled cutaneous and internal organs fibrosis. Diagnosis of SSc in an early phase can be difficult because of a lack of typical symptoms. The delay in diagnosis and treatment of SSc may lead to uncontrolled progression of the disease, thus identification of possible early indicators of skin and organ involvement to prevent their further damage is necessary. The aim of this study is to review the latest biomarkers of organ involvement in SSc. In patients with lung fibrosis lung-epithelial-derived surfactant protein (SP-D), the glycoprotein Krebs von den Lungen-6 (KL-6), and chemokine ligands 2, 4 and 18 (CCL2, CXCL4, CCL18) are elevated, while in patients with skin fibrosis serum levels of heat shock protein 27 (Hsp27), interleukin 16 (IL-16), and IgG-galactosylation ratio are increased. Adiponectin concentration is inversely correlated with the intensity of cutaneous fibrosis. Skin gene profiling also seems very promising. In patients with heart involvement increased serum levels of brain natriuretic peptide (BNP) are present, as well as raised Midkine and Follistatin-like 3 (FSTL3) proteins, ratios of Cu/Se and ceruloplasmin(CP) /Circulating selenoprotein P(SELENOP) and higher whole blood viscosity level. Elevated calprotectin levels are found in individuals with gastrointestinal involvement. Increased levels of chemerin and ARA autoantibodies are associated with renal involvement, whereas high levels of adhesion molecules are found in patients with scleroderma renal crisis (SRC). Currently there are no biomarkers in use that can specifically identify the early involvement of organs.
系统性硬化症（SSc）是一种自身免疫性疾病，其特征为血管病变以及皮肤和内脏器官的纤维化不受控制。由于缺乏典型症状，早期诊断SSc可能会很困难。SSc诊断和治疗的延迟可能导致疾病不受控制地进展，因此识别皮肤和器官受累的可能早期指标以防止其进一步损害是必要的。本研究的目的是综述SSc器官受累的最新生物标志物。在肺纤维化患者中，肺上皮来源的表面活性蛋白（SP-D）、糖蛋白克雷布斯冯登肺-6（KL-6）以及趋化因子配体2、4和18（CCL2、CXCL4、CCL18）水平升高，而在皮肤纤维化患者中，血清热休克蛋白27（Hsp27）、白细胞介素16（IL-16）水平以及IgG-半乳糖基化比率升高。脂联素浓度与皮肤纤维化程度呈负相关。皮肤基因谱分析似乎也很有前景。在心脏受累患者中，血清脑钠肽（BNP）水平升高，同时Midkine和类卵泡抑素3（FSTL3）蛋白、铜/硒比率以及铜蓝蛋白（CP）/循环硒蛋白P（SELENOP）升高，全血粘度水平也更高。在胃肠道受累个体中发现钙卫蛋白水平升高。趋化素和抗合成酶抗体（ARA）水平升高与肾脏受累有关，而在硬皮病肾危象（SRC）患者中发现高水平的黏附分子。目前尚无可用的生物标志物能够特异性识别器官的早期受累情况。
