Abstract：
OBJECTIVE: To develop evidence-based recommendations for the non-pharmacological and pharmacological management of Raynaud\'s phenomenon (RP) and digital ulcers (DUs) in patients with systemic sclerosis and other immune-mediated connective tissue diseases (CTDs).
METHODS: A task force comprising 21 rheumatologists, two surgeons (vascular and plastic), two nurses, and one patient representative was established. Following a systematic literature review performed to inform the recommendations, statements were formulated and discussed during two meetings (one online and one in-person). Levels of evidence, grades of recommendation (GoR), and level of agreement (LoA) were determined.
RESULTS: Five overarching principles and 13 recommendations were developed. GoR ranged from A to D. The mean ± standard difference (SD) LoA with the overarching principles and recommendations ranged from 7.8±2.1 to 9.8±0.4. Briefly, the management of RP and DUs in patients with CTDs should be coordinated by a multidisciplinary team and based on shared decisions with patients. Nifedipine should be used as first-line therapy for RP and/or DUs. Sildenafil, tadalafil, and/or iloprost IV are second-line options for severe and/or refractory patients with RP and/or DUs. Sildenafil, tadalafil and/or Iloprost IV, should be prescribed for healing and prevention (also including bosentan) of DUs. In patients with RP and/or DUs, non-pharmacological interventions might be considered as add-ons, but there is limited quality and quantity of scientific evidence supporting their use.
CONCLUSIONS: These recommendations will inform rheumatologists, specialist nurses, other healthcare professionals, and patients about a comprehensive and personalized management of RP and DUs. A research agenda was developed to address unmet needs, particularly for non-pharmacologic interventions.
METHODS: A task force comprising 21 rheumatologists, two surgeons (vascular and plastic), two nurses, and one patient representative was established. Following a systematic literature review performed to inform the recommendations, statements were formulated and discussed during two meetings (one online and one in-person). Levels of evidence, grades of recommendation (GoR), and level of agreement (LoA) were determined.
RESULTS: Five overarching principles and 13 recommendations were developed. GoR ranged from A to D. The mean ± standard difference (SD) LoA with the overarching principles and recommendations ranged from 7.8±2.1 to 9.8±0.4. Briefly, the management of RP and DUs in patients with CTDs should be coordinated by a multidisciplinary team and based on shared decisions with patients. Nifedipine should be used as first-line therapy for RP and/or DUs. Sildenafil, tadalafil, and/or iloprost IV are second-line options for severe and/or refractory patients with RP and/or DUs. Sildenafil, tadalafil and/or Iloprost IV, should be prescribed for healing and prevention (also including bosentan) of DUs. In patients with RP and/or DUs, non-pharmacological interventions might be considered as add-ons, but there is limited quality and quantity of scientific evidence supporting their use.
CONCLUSIONS: These recommendations will inform rheumatologists, specialist nurses, other healthcare professionals, and patients about a comprehensive and personalized management of RP and DUs. A research agenda was developed to address unmet needs, particularly for non-pharmacologic interventions.
摘要：
目的：为系统性硬化症和其他免疫介导的结缔组织疾病（CTD）患者的雷诺现象（RP）和数字溃疡（DU）的非药物和药物治疗提供循证建议。
方法：由21名风湿病专家组成的工作组，两名外科医生（血管和整形），两个护士,并建立了一名患者代表。在进行系统的文献综述以告知建议后，在两次会议(一次在线，一次面对面)上制定和讨论了声明。证据水平,推荐等级(GoR)，并确定了协议水平(LoA)。
结果：制定了五项总体原则和13项建议。GoR范围从A到D。具有总体原则和建议的平均值±标准差（SD）LoA范围从7.8±2.1到9.8±0.4。简而言之，CTDs患者RP和DUs的管理应由多学科团队协调,并基于与患者的共同决策.硝苯地平应用作RP和/或DU的一线治疗。西地那非,他达拉非,和/或伊洛前列素IV是严重和/或难治性RP和/或DU患者的二线选择。西地那非,他达拉非和/或伊洛前列素IV，应规定用于治疗和预防（包括波生坦）的DU。在RP和/或DU患者中，非药物干预可能被认为是附加的，但是支持其使用的科学证据的质量和数量有限。
结论：这些建议将告知风湿病学家，专科护士,其他医疗保健专业人员，和患者关于RP和DU的全面和个性化管理。制定了一项研究议程，以解决未满足的需求，特别是对于非药物干预。
方法：由21名风湿病专家组成的工作组，两名外科医生（血管和整形），两个护士,并建立了一名患者代表。在进行系统的文献综述以告知建议后，在两次会议(一次在线，一次面对面)上制定和讨论了声明。证据水平,推荐等级(GoR)，并确定了协议水平(LoA)。
结果：制定了五项总体原则和13项建议。GoR范围从A到D。具有总体原则和建议的平均值±标准差（SD）LoA范围从7.8±2.1到9.8±0.4。简而言之，CTDs患者RP和DUs的管理应由多学科团队协调,并基于与患者的共同决策.硝苯地平应用作RP和/或DU的一线治疗。西地那非,他达拉非,和/或伊洛前列素IV是严重和/或难治性RP和/或DU患者的二线选择。西地那非,他达拉非和/或伊洛前列素IV，应规定用于治疗和预防（包括波生坦）的DU。在RP和/或DU患者中，非药物干预可能被认为是附加的，但是支持其使用的科学证据的质量和数量有限。
结论：这些建议将告知风湿病学家，专科护士,其他医疗保健专业人员，和患者关于RP和DU的全面和个性化管理。制定了一项研究议程，以解决未满足的需求，特别是对于非药物干预。
