Akter Tanjina, Silver Richard M, Bogatkevich Galina S
Division of Rheumatology and Immunology, Department of Medicine, Medical University of South Carolina, 114 Doughty Street, Charleston, SC, 29425, USA,
Curr Rheumatol Rep. 2014 Apr;16(4):411. doi: 10.1007/s11926-014-0411-1.
Systemic sclerosis (scleroderma, SSc) is a heterogeneous autoimmune connective tissue disease of unknown etiology. Interstitial lung disease (ILD) is a frequent complication, and a significant contributor to morbidity and mortality among SSc patients. SSc-ILD most commonly occurs within 10 years of diagnosis, and may be seen in patients with either the limited or diffuse cutaneous subset of SSc. SSc-ILD is a multifaceted disease process in which different factors and pathways are involved. Aberrant function of a variety of lung cells, cytokines, growth factors, peptides, and bioactive proteins, in combination with genetic and epigenetic regulators, have crucial functions in the pathogenesis of this disease. Here we present our view on recent advances regarding the pathogenesis of SSc-ILD.
系统性硬化症（硬皮病，SSc）是一种病因不明的异质性自身免疫性结缔组织疾病。间质性肺疾病（ILD）是一种常见的并发症，也是SSc患者发病和死亡的重要原因。SSc-ILD最常发生在诊断后的10年内，在局限性或弥漫性皮肤亚型的SSc患者中均可出现。SSc-ILD是一个多方面的疾病过程，涉及不同的因素和途径。多种肺细胞、细胞因子、生长因子、肽和生物活性蛋白的异常功能，与遗传和表观遗传调节因子相结合，在该疾病的发病机制中具有关键作用。在此，我们阐述对SSc-ILD发病机制最新进展的看法。
