Division of Allergy-Immunology-Rheumatology, Department of Medicine, Faculty of Medicine, Khon Kaen University, Khon Kaen, Thailand.
Clin Rheumatol. 2011 Sep;30(9):1235-40. doi: 10.1007/s10067-011-1744-z. Epub 2011 Apr 12.
Skin tightness is a major clinical manifestation of systemic sclerosis (SSc). Importantly for both clinicians and patients, spontaneous regression of the fibrosis process has been documented. The purpose of this study is to identify the incidence and related clinical characteristics of spontaneous regression among Thai SSc patients. A historical cohort with 4 years of follow-up was performed among SSc patients over 15 years of age diagnosed with SSc between January 1, 2005 and December 31, 2006 in Khon Kaen, Thailand. The start date was the date of the first symptom and the end date was the date of the skin score ≤2. To estimate the respective probability of regression and to assess the associated factors, the Kaplan-Meier method and Cox regression analysis was used. One hundred seventeen cases of SSc were included with a female to male ratio of 1.5:1. Thirteen patients (11.1%) experienced regression. The incidence rate of spontaneous skin regression was 0.31 per 100 person-months and the average duration of SSc at the time of regression was 35.9±15.6 months (range, 15.7-60 months). The factors that negatively correlated with regression were (a) diffuse cutaneous type, (b) Raynaud's phenomenon, (c) esophageal dysmotility, and (d) colchicine treatment at onset with a respective hazard ratio (HR) of 0.19, 0.19, 0.26, and 0.20. By contrast, the factor that positively correlated with regression was active alveolitis with cyclophosphamide therapy at onset with an HR of 4.23 (95% CI, 1.23-14.10). After regression analysis, only Raynaud's phenomenon at onset and diffuse cutaneous type had a significantly negative correlation to regression. A spontaneous regression of the skin fibrosis process was not uncommon among Thai SSc patients. The factors suggesting a poor predictor for cutaneous manifestation were Raynaud's phenomenon, diffuse cutaneous type while early cyclophosphamide therapy might be related to a better skin outcome.
皮肤紧绷是系统性硬化症（SSc）的主要临床表现。重要的是，对于临床医生和患者来说，纤维化过程的自发消退已经得到证实。本研究的目的是确定泰国 SSc 患者自发消退的发生率和相关临床特征。在泰国孔敬，对 2005 年 1 月 1 日至 2006 年 12 月 31 日期间被诊断为 SSc 的年龄在 15 岁以上的 SSc 患者进行了为期 4 年的历史队列研究。起始日期为第一个症状出现的日期，截止日期为皮肤评分≤2 的日期。为了估计各自的回归概率并评估相关因素，使用 Kaplan-Meier 方法和 Cox 回归分析。共纳入 117 例 SSc 患者，男女比例为 1.5:1。13 例（11.1%）患者出现消退。自发性皮肤消退的发生率为 0.31/100 人-月，消退时 SSc 的平均持续时间为 35.9±15.6 个月（范围 15.7-60 个月）。与消退负相关的因素为（a）弥漫性皮肤型，（b）雷诺现象，（c）食管运动障碍和（d）在发病时使用秋水仙碱治疗，相应的风险比（HR）为 0.19、0.19、0.26 和 0.20。相比之下，与消退正相关的因素是在发病时用环磷酰胺治疗的活动性肺泡炎，其 HR 为 4.23（95%CI，1.23-14.10）。在回归分析后，只有发病时的雷诺现象和弥漫性皮肤型与消退有显著的负相关。泰国 SSc 患者的皮肤纤维化过程自发消退并不少见。提示皮肤表现不良预测因素的因素有雷诺现象、弥漫性皮肤型，而早期使用环磷酰胺治疗可能与更好的皮肤结局相关。
