Abstract：
BACKGROUND: Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc.
METHODS: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed.
RESULTS: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud\'s phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found.
CONCLUSIONS: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice.
METHODS: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed.
RESULTS: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud\'s phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found.
CONCLUSIONS: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice.
摘要：
背景：系统性硬化症（SSc）是一种罕见的具有异质性表现的慢性自身免疫性疾病。在过去的十年里，已经进行了几项临床试验来评估SSc的新治疗方案.这项工作的目的是根据可用于SSc药理学管理的新证据更新巴西风湿病学会的建议。
方法：系统综述，包括根据患者/人群阐述的预定义问题的随机临床试验（RCT），干预,比较,和结果（PICO）战略进行。可用证据的评级是根据建议评估的等级进行的，开发和评估(等级)方法。成为一个推荐，至少需要75%的投票小组同意。
结果：阐述了关于雷诺现象的药物治疗的六项建议，治疗（愈合）和预防数字溃疡，皮肤受累,根据RCT的结果，SSc患者的间质性肺病（ILD）和胃肠道受累。新药,如利妥昔单抗，作为皮肤受累的治疗选择，利妥昔单抗,托珠单抗和尼达尼布被纳入ILD的治疗方案.根据投票小组的专家意见，详细阐述了硬皮病肾危象和肌肉骨骼受累的药物治疗建议，因为没有发现安慰剂对照的随机对照试验。
结论：本指南根据文献证据和专家意见更新并纳入新的SSc治疗方案。为临床实践决策提供支持。
方法：系统综述，包括根据患者/人群阐述的预定义问题的随机临床试验（RCT），干预,比较,和结果（PICO）战略进行。可用证据的评级是根据建议评估的等级进行的，开发和评估(等级)方法。成为一个推荐，至少需要75%的投票小组同意。
结果：阐述了关于雷诺现象的药物治疗的六项建议，治疗（愈合）和预防数字溃疡，皮肤受累,根据RCT的结果，SSc患者的间质性肺病（ILD）和胃肠道受累。新药,如利妥昔单抗，作为皮肤受累的治疗选择，利妥昔单抗,托珠单抗和尼达尼布被纳入ILD的治疗方案.根据投票小组的专家意见，详细阐述了硬皮病肾危象和肌肉骨骼受累的药物治疗建议，因为没有发现安慰剂对照的随机对照试验。
结论：本指南根据文献证据和专家意见更新并纳入新的SSc治疗方案。为临床实践决策提供支持。
