University of Western Ontario, London, ON, Canada.
Clin Exp Rheumatol. 2012 Mar-Apr;30(2 Suppl 71):S38-43. Epub 2012 May 29.
There is currently no consensus on best practice in systemic sclerosis (SSc). To determine if variability in treatment and investigations exists, practices among Canadian Sclerodermia Research Group (CSRG) centres were compared.
Prospective clinical and demographic data from adult SSc patients are collected annually from 15 CSRG treatment centres. Laboratory parameters, self-reported socio-demographic questionnaires, current and past medications and disease outcome measures are recorded. For centres with >50 patients enrolled, treatment practices were analysed to determine practice variability.
Data from 640 of 938 patients within the CSRG database met inclusion criteria, where 87.3% were female, the mean ± SEM age was 55.3±0.5, 48.9% had limited SSc and 47.8% had diffuse SSc (and 3.3% uncharacterised). Some investigation and treatment practices were inconsistent among 6 centres including proportion receiving: PDE5 (phosphodiesterase type 5) inhibitors for Raynaud's phenomenon (p=0.036); cyclophosphamide (p=0.037) and azathioprine (p=0.037) for treatment of ILD; and current use of D-penicillamine, although uncommon, varied among sites. Annual echocardiograms and PFTs were frequently done and did not vary among sites but the rate of pulmonary arterial hypertension (PAH) was directly related to site size and this was not the case for other organ involvement.
Despite routine tests within a database, site variation in SSc with respect to investigations and management among CSRG centres exists suggesting a need for a standardised approach to the investigation and treatment of SSc. One can speculate that larger centres are more export in detecting PAH.
目前对于硬皮病（SSc）尚无最佳治疗共识。为了确定治疗和检查是否存在差异，我们比较了加拿大硬皮病研究组（CSRG）中心的实践。
每年从 15 个 CSRG 治疗中心收集成人 SSc 患者的前瞻性临床和人口统计学数据。记录实验室参数、自我报告的社会人口学问卷、当前和过去的药物以及疾病结局指标。对于纳入患者超过 50 人的中心，分析治疗实践以确定实践差异。
CSRG 数据库中符合纳入标准的 640 名患者中，87.3%为女性，平均年龄±SEM 为 55.3±0.5 岁，48.9%为局限型 SSc，47.8%为弥漫型 SSc（3.3%为未定型）。6 个中心中，一些检查和治疗方法存在差异，包括接受磷酸二酯酶 5 型（PDE5）抑制剂治疗雷诺现象的比例（p=0.036）；接受环磷酰胺（p=0.037）和硫唑嘌呤（p=0.037）治疗ILD 的比例；以及目前使用青霉胺的比例，尽管不太常见，但各中心之间存在差异。每年进行的超声心动图和 PFT 检查较为频繁，但各中心之间没有差异，但肺动脉高压（PAH）的发生率与中心规模直接相关，而其他器官受累则并非如此。
尽管在数据库中进行了常规检查，但 CSRG 中心的 SSc 在检查和管理方面存在差异，这表明需要对 SSc 的检查和治疗采用标准化方法。可以推测，较大的中心更善于检测 PAH。
