Akimoto S, Ishikawa O, Muro Y, Takagi H, Tamura T, Miyachi Y
Department of Dermatology, Gunma University School of Medicine, Japan.
J Dermatol. 1999 Jan;26(1):18-22. doi: 10.1111/j.1346-8138.1999.tb03503.x.
Limited cutaneous systemic sclerosis (lSSc) or CREST syndrome is sometimes complicated by primary biliary cirrhosis (PBC). To characterize the clinical and immunological features of patients with SSc overlapping PBC (SSc-PBC), the clinical and laboratory data of 11 SSc-PBC were studied. Since all of the SSc-PBC were lSSc, the features of SSc-PBC were compared with those of 266 patients with lSSc alone. The sera from SSc-PBC showed a significantly higher positivity for anti-centromere antibody (ACA) (p < 0.0005) and higher reactivity to centromere protein-C. Although SSc-PBC more frequently exhibited calcinosis and telangiectasia (p < 0.05), there was no statistical difference in other features of CREST syndrome. There was no statistical difference in other scleroderma-related features either. SSc-PBC were more frequently complicated with Sjögren syndrome (p < 0.05). The most prominent feature of SSc-PBC was a higher incidence of ACA than in lSSc alone.
局限性皮肤型系统性硬化症（lSSc）或CREST综合征有时会并发原发性胆汁性肝硬化（PBC）。为了描述系统性硬化症合并PBC（SSc-PBC）患者的临床和免疫学特征，对11例SSc-PBC患者的临床和实验室数据进行了研究。由于所有SSc-PBC患者均为lSSc，因此将SSc-PBC患者的特征与266例单纯lSSc患者的特征进行了比较。SSc-PBC患者的血清抗着丝点抗体（ACA）阳性率显著更高（p < 0.0005），且对着丝点蛋白C的反应性更高。尽管SSc-PBC患者更常出现钙质沉着和毛细血管扩张（p < 0.05），但CREST综合征的其他特征无统计学差异。其他与硬皮病相关的特征也无统计学差异。SSc-PBC患者更常并发干燥综合征（p < 0.05）。SSc-PBC最突出的特征是ACA的发生率高于单纯lSSc患者。
