Valentini Gabriele, Marcoccia Antonella, Cuomo Giovanna, Vettori Serena, Iudici Michele, Bondanini Francesco, Santoriello Carlo, Ciani Aldo, Cozzolino Domenico, De Matteis Giovanni Maria, Cappabianca Salvatore, Vitelli Filiberto, Spanò Alberto
Arthritis Res Ther. 2013;15(3):R63. doi: 10.1186/ar4236.
Early systemic sclerosis (SSc) is characterized by Raynaud's phenomenon together with scleroderma marker autoantibodies and/or a scleroderma pattern at capillaroscopy and no other distinctive feature of SSc. Patients presenting with marker autoantibodies plus a capillaroscopic scleroderma pattern seem to evolve into definite SSc more frequently than patients with either feature. Whether early SSc patients with only marker autoantibodies or capillaroscopic positivity differ in any aspect at presentation is unclear.
Seventy-one consecutive early SSc patients were investigated for preclinical cardiopulmonary alterations. Out of these, 44 patients and 25 controls affected by osteoarthritis or primary fibromyalgia syndrome were also investigated for serum markers of fibroblast (carboxyterminal propeptide of collagen I), endothelial (soluble E-selectin) and T-cell (soluble IL-2 receptor alpha) activation.
Thirty-two of the 71 patients (45.1%) had both a marker autoantibody and a capillaroscopic scleroderma pattern (subset 1), 16 patients (22.5%) had only a marker autoantibody (subset 2), and 23 patients (32.4%) had only a capillaroscopic scleroderma pattern (subset 3). Patients with marker autoantibodies (n = 48, 67.6%) had a higher prevalence of impaired diffusing lung capacity for carbon monoxide (P = 0.0217) and increased serum levels of carboxyterminal propeptide of collagen I (P = 0.0037), regardless of capillaroscopic alterations. Patients with a capillaroscopic scleroderma pattern (n = 55, 77.5%) had a higher prevalence of puffy fingers (P = 0.0001) and increased serum levels of soluble E-selectin (P = 0.0003) regardless of marker autoantibodies.
These results suggest that the autoantibody and microvascular patterns in early SSc may each be related to different clinical-preclinical features and circulating activation markers at presentation. Longitudinal studies are warranted to investigate whether these subsets undergo a different disease course over time.
早期系统性硬化症（SSc）的特征为雷诺现象，伴有硬皮病标志物自身抗体和/或毛细血管镜检查显示的硬皮病样改变，且无SSc的其他明显特征。出现标志物自身抗体加毛细血管镜硬皮病样改变的患者似乎比仅具有其中任一特征的患者更易发展为明确的SSc。仅具有标志物自身抗体或毛细血管镜检查呈阳性的早期SSc患者在临床表现的任何方面是否存在差异尚不清楚。
对71例连续的早期SSc患者进行临床前心肺改变的研究。其中，44例患者以及25例患骨关节炎或原发性纤维肌痛综合征的对照者还接受了成纤维细胞（I型胶原羧基末端前肽）、内皮细胞（可溶性E选择素）和T细胞（可溶性白细胞介素-2受体α）活化血清标志物的检测。
71例患者中，32例（45.1%）同时具有标志物自身抗体和毛细血管镜硬皮病样改变（亚组1），16例患者（22.5%）仅具有标志物自身抗体（亚组2），23例患者（32.4%）仅具有毛细血管镜硬皮病样改变（亚组3）。具有标志物自身抗体的患者（n = 48，67.6%）一氧化碳弥散功能受损的患病率较高（P = 0.0217），且I型胶原羧基末端前肽血清水平升高（P = 0.0037），与毛细血管镜改变无关。具有毛细血管镜硬皮病样改变的患者（n = 55，77.5%）手指肿胀的患病率较高（P = 0.0001），且可溶性E选择素血清水平升高（P = 0.0003），与标志物自身抗体无关。
这些结果表明，早期SSc中的自身抗体和微血管模式可能各自与临床表现时不同的临床 - 临床前特征及循环活化标志物相关。有必要进行纵向研究以调查这些亚组随时间推移是否经历不同的疾病进程。
