Tânia Santiago, Ana Catarina Duarte, Alexandre Sepriano, Alice Castro, Bruno Rosa, Catarina Resende, Daniela Oliveira, Eduardo Dourado, Emanuel Costa, Filipe Cunha-Santos, Georgina Terroso, Gonçalo Boleto, Ivone Silva, Lurdes Barbosa, Joana Silva, Joana Sousa Neves, Maria João Salvador, Maria João Gonçalves, Miguel Gomes Guerra, Raquel Miriam Ferreira, Rúben Duarte-Fernandes, Sofia Barreira, Vítor Silvestre Teixeira, Ana Lúcia Tomás, Vasco Romão, Ana Cordeiro
{"title":"Portuguese Recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases.","authors":"Tânia Santiago, Ana Catarina Duarte, Alexandre Sepriano, Alice Castro, Bruno Rosa, Catarina Resende, Daniela Oliveira, Eduardo Dourado, Emanuel Costa, Filipe Cunha-Santos, Georgina Terroso, Gonçalo Boleto, Ivone Silva, Lurdes Barbosa, Joana Silva, Joana Sousa Neves, Maria João Salvador, Maria João Gonçalves, Miguel Gomes Guerra, Raquel Miriam Ferreira, Rúben Duarte-Fernandes, Sofia Barreira, Vítor Silvestre Teixeira, Ana Lúcia Tomás, Vasco Romão, Ana Cordeiro","doi":"","DOIUrl":null,"url":null,"abstract":"<p><strong>Objective: </strong>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).</p><p><strong>Methods: </strong>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.</p><p><strong>Results: </strong>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.</p><p><strong>Conclusions: </strong>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.</p>","PeriodicalId":29669,"journal":{"name":"ARP Rheumatology","volume":"3 2","pages":"84-94"},"PeriodicalIF":1.4000,"publicationDate":"2024-04-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":null,"platform":"Semanticscholar","paperid":null,"PeriodicalName":"ARP Rheumatology","FirstCategoryId":"1085","ListUrlMain":"","RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"Q3","JCRName":"RHEUMATOLOGY","Score":null,"Total":0}
引用次数: 0
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.
目的为系统性硬化症和其他免疫介导结缔组织病(CTD)患者雷诺现象(RP)和数字溃疡(DU)的非药物和药物治疗制定循证建议:成立了一个由 21 名风湿病专家、两名外科医生(血管外科医生和整形外科医生)、两名护士和一名患者代表组成的工作组。在进行了系统的文献回顾以提供建议信息后,在两次会议(一次在线会议和一次现场会议)上制定并讨论了声明。确定了证据等级、建议等级(GoR)和同意等级(LoA):结果:制定了五项总体原则和 13 项建议。总体原则和建议的平均±标准差 (SD) LoA 为 7.8±2.1 到 9.8±0.4。简而言之,CTD 患者的 RP 和 DU 管理应由多学科团队协调,并与患者共同决策。硝苯地平应作为 RP 和/或 DU 的一线治疗药物。西地那非、他达拉非和/或伊洛前列素静脉注射是严重和/或难治性 RP 和/或 DU 患者的二线选择。西地那非、他达拉非和/或伊洛前列素静脉滴注应作为治疗和预防(也包括波生坦)DU 的处方。对于RP和/或DUs患者,非药物干预措施可作为附加治疗,但支持其使用的科学证据在质量和数量上都很有限:这些建议将为风湿免疫科医生、专科护士、其他医疗保健专业人员和患者提供全面、个性化的 RP 和 DUs 管理方法。针对尚未满足的需求,特别是非药物干预方面的需求,制定了一项研究议程。