Department of Medicine, Division of Rheumatology, Hospital for Special Surgery, 535 East 70th Street, New York, NY, 10021, USA.
Curr Rheumatol Rep. 2020 Nov 26;23(1):3. doi: 10.1007/s11926-020-00970-z.
Systemic sclerosis (SSc) is a life-threatening autoimmune disease that causes debilitating skin fibrosis. The skin in SSc is easily accessible, and skin biopsies may provide rich biological data regarding underlying disease pathophysiology. Here, we review literature relevant to the potential for skin histology to serve as a diagnostic, pharmacokinetic/response, and predictive biomarker in SSc.
Multiple histologic parameters correlate with SSc severity, including alpha smooth muscle actin (aSMA), CD34, collagen density, thickness, and inflammatory cell infiltration. Recent clinical trials incorporate skin histology as exploratory outcome measurements; however, a standard approach is not yet established. The possibility that skin histology may be useful as a predictive biomarker was suggested by a recent study that identified genes related to skin aSMA and CD34 staining intensity that were increased at baseline among improvers versus nonimprovers. Current literature supports skin histology as a mechanism to measure treatment response, but future work is needed to define minimally meaningful changes in key SSc skin histologic features.
系统性硬化症（SSc）是一种危及生命的自身免疫性疾病，可导致衰弱性皮肤纤维化。SSc 的皮肤很容易接近，皮肤活检可能提供有关潜在疾病病理生理学的丰富生物学数据。在这里，我们回顾了与皮肤组织病理学作为 SSc 的诊断、药代动力学/反应和预测生物标志物的潜在用途相关的文献。
多种组织学参数与 SSc 的严重程度相关，包括α平滑肌肌动蛋白（αSMA）、CD34、胶原密度、厚度和炎症细胞浸润。最近的临床试验将皮肤组织病理学作为探索性的结局测量方法纳入其中；然而，尚未建立标准方法。最近的一项研究表明，皮肤组织病理学可能作为预测生物标志物有用，该研究发现与皮肤 αSMA 和 CD34 染色强度相关的基因在改善者与非改善者之间基线时增加，这些基因与改善者与非改善者有关。目前的文献支持皮肤组织病理学作为测量治疗反应的一种机制，但需要进一步的工作来定义 SSc 皮肤组织病理学特征中具有最小意义的变化。
