Niklas Karolina, Sikorska Dorota, Mularek-Kubzdela Tatiana, Witoszyńska-Sobkowiak Joanna, Żychowska Iwona, Samborski Włodzimierz
Department of Rheumatology and Rehabilitation and Internal Diseases, Poznan University of Medical Science, 28 Czerwca 1956r. 135/147, 61-545 Poznan, Poland.
Department of Cardiology, Poznan University of Medical Sciences, Długa 1/2, 60-848 Poznan, Poland.
Int J Mol Sci. 2025 Jun 13;26(12):5684. doi: 10.3390/ijms26125684.
The most serious complications of systemic sclerosis (SSc) and mixed connective tissue disease (MCTD) include lung fibrosis (LF) and pulmonary hypertension (PH). The aim of this study was to find any association between the serological profile and the incidence of these complications. The tested group included 121 persons (87 SSc, 34 MCTD); mean age 55.6 ± 13.4 years. Patients were qualified for the LF presence group based on HRCT. Likelihood of PH was determined using echocardiography. The presence of antinuclear antibodies (ANA) was assessed using indirect immunofluorescence, ANA-profile, sclerosis-profile (using EUROIMMUN kits), and antiphospholipid antibodies (aPL) (using the ELISA method). Distribution of individual antibody types was at a level similar to the previously described groups in the Polish population and differed from the American and African population. A positive correlation was found between LF and the presence of anti-Scl-70 ( = 0.024) antibodies, negative correlation was found between LF and the presence of anti-histone ( = 0.03), anti-centromere A ( = 0.009), anti-centromere B ( = 0.014), and anti-nucleosomes ( = 0.03) antibodies. No correlation between the presence of aPL and the above complications was found. The prevalence of individual antibody types in SSc and MCTD may have ethnic and geographical grounds. Scl-70 antibodies correlate positively with LF. Anti-centromere, anti-histone, and anti-nucleosome antibodies reduce its risk. No correlation between aPL and the occurrence of LF and elevated PH risk was found.
系统性硬化症(SSc)和混合性结缔组织病(MCTD)最严重的并发症包括肺纤维化(LF)和肺动脉高压(PH)。本研究的目的是找出血清学特征与这些并发症发生率之间的任何关联。测试组包括121人(87例SSc,34例MCTD);平均年龄55.6±13.4岁。根据高分辨率计算机断层扫描(HRCT)将患者纳入LF存在组。使用超声心动图确定PH的可能性。使用间接免疫荧光、ANA谱、硬化谱(使用EUROIMMUN试剂盒)和抗磷脂抗体(aPL)(使用酶联免疫吸附测定法(ELISA))评估抗核抗体(ANA)的存在。个体抗体类型的分布与波兰人群中先前描述的组处于相似水平,与美国和非洲人群不同。发现LF与抗Scl-70(=0.024)抗体的存在呈正相关,LF与抗组蛋白(=0.03)、抗着丝粒A(=0.009)、抗着丝粒B(=0.014)和抗核小体(=0.03)抗体的存在呈负相关。未发现aPL的存在与上述并发症之间存在相关性。SSc和MCTD中个体抗体类型的流行率可能有种族和地理原因。Scl-70抗体与LF呈正相关。抗着丝粒、抗组蛋白和抗核小体抗体可降低其风险。未发现aPL与LF的发生和PH风险升高之间存在相关性。