Nagy Lőrinc, Nagy Gábor, Juhász Tamás, Fillér Csaba, Szűcs Gabriella, Szekanecz Zoltán, Vereb György, Antal-Szalmás Péter, Szöőr Árpád
Department of Biophysics and Cell Biology, Faculty of Medicine, University of Debrecen, H-4032 Debrecen, Hungary.
Department of Laboratory Medicine, Faculty of Medicine, University of Debrecen, H-4032 Debrecen, Hungary.
Int J Mol Sci. 2025 Mar 14;26(6):2618. doi: 10.3390/ijms26062618.
Systemic sclerosis (SSc) is a complex autoimmune disease characterized by fibrosis, immune dysregulation, and vascular dysfunction, yet its pathogenesis remains incompletely understood. This study compares two widely used animal models of SSc-the bleomycin-induced fibrosis model and the collagen-V-induced autoimmune model-to evaluate their ability to replicate key disease features. In the bleomycin model, consistent cardiac fibrosis was observed across treatment groups despite variability in fibrosis in the skin and lungs, suggesting organ-specific differences in susceptibility. The collagen-V model demonstrated robust autoantibody production against collagen-V, confirming its utility in studying immune activation, though fibrosis was largely confined to the heart. While the bleomycin model excels at mimicking rapid fibrosis and is suitable for testing antifibrotic therapies, the collagen-V model provides insights into antigen-specific autoimmunity. Both models highlight the dynamic nature of fibrosis, where ECM deposition and degradation occur concurrently, complicating its use as a quantitative disease marker. Cardiac fibrosis emerged as a consistent feature in both models, emphasizing its relevance in SSc pathophysiology. Combining these models or refining their design through hybrid approaches, extended timelines, or sex and age adjustments could enhance their translational utility. These findings advance understanding of SSc mechanisms and inform therapeutic development for this challenging disease.
系统性硬化症(SSc)是一种复杂的自身免疫性疾病,其特征为纤维化、免疫失调和血管功能障碍,但其发病机制仍未完全明确。本研究比较了两种广泛应用的SSc动物模型——博来霉素诱导的纤维化模型和胶原蛋白V诱导的自身免疫模型——以评估它们复制关键疾病特征的能力。在博来霉素模型中,尽管皮肤和肺部的纤维化存在差异,但各治疗组均观察到一致的心脏纤维化,提示易感性存在器官特异性差异。胶原蛋白V模型显示出针对胶原蛋白V的强大自身抗体产生,证实了其在研究免疫激活方面的效用,尽管纤维化主要局限于心脏。虽然博来霉素模型在模拟快速纤维化方面表现出色,适用于测试抗纤维化疗法,但胶原蛋白V模型为抗原特异性自身免疫提供了见解。两种模型都突出了纤维化的动态性质,即细胞外基质沉积和降解同时发生,这使得其作为定量疾病标志物的应用变得复杂。心脏纤维化在两种模型中均表现为一致特征,强调了其在SSc病理生理学中的相关性。结合这些模型或通过混合方法、延长时间线或进行性别和年龄调整来优化其设计,可能会提高它们的转化效用。这些发现增进了对SSc机制的理解,并为这种具有挑战性的疾病的治疗发展提供了信息。