Portuguese Recommendations for the management of Raynaud’s phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases
Authors:   Rheumatology Department, Unidade Local de Saúde de Coimbra - Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal, Santiago T ,   Faculty of Medicine, Coimbra University, Duarte AC ,   Rheumatology Department, Unidade Local de Saúde Almada- Seixal - Hospital Garcia de Orta, Almada, Portugal, Sepriano A ,   Rheumatology Department, Hospital de Egas Moniz, Unidade Local de Saúde de Lisboa Ocidental, Lisboa, Portugal; CEDOC, NOVA Medical School, Universidade Nova de Lisboa, Portugal, Castro A ,   Rheumatology Department, Unidade Local de Saúde Almada- Seixal - Hospital Garcia de Orta, Almada, Portugal, Rosa B ,   Plastic Surgery Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Portugal, Resende C ,   Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal, Oliveira D ,   Rheumatology Department, Unidade Local de Saúde de São João, Porto, Portugal; 9 Center for Health Technology and Services Research (CINTESIS), Faculty of Medicine, University of Porto, Porto, Portugal;, Dourado D ,   Rheumatology Department, Unidade Local de Saúde da Região de Aveiro, Aveiro, Portugal. 11 Aveiro Rheumatology Research Centre, Egas Moniz Health Alliance, Aveiro, Portugal; 12 Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina da Universidade de Lisboa, Costa E ,   Rheumatology Department, Unidade Local de Saúde de Braga, Braga, Portugal, Cunha-Santos F ,   Rheumatology Department, Unidade Local de Saúde da Guarda – Hospital Sousa Martins, Guarda, Portugal, Terroso G ,   Rheumatology Department, Unidade Local de Saúde de São João, Porto, Portugal; 9 Center for Health Technology and Services Research (CINTESIS), Faculty of Medicine, University of Porto, Porto, Portugal, Boleto G ,   Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal, Silva I ,   Serviço de Angiologia e Cirurgia Vascular, Centro Hospitalar Universitário do Porto; Unit for Multidisciplinary Research in Biomedicine, ICBAS – School of medicine and biomedical Sciences, University of Porto, Porto, Portugal; ITR – Laboratory for Integrative and Translational Research in Population Health, Porto Portugal, Barbosa L ,   Rheumatology Department, Unidade Local de Saúde Almada- Seixal - Hospital Garcia de Orta, Almada, Portugal, Silva J ,   Rheumatology Department, ULS Braga, Braga, Portugal, Sousa Neves J ,   Rheumatology Department, ULS Braga, Braga, Portugal, Salvador MJ ,   Rheumatology Department, Unidade Local de Saúde de Coimbra - Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal; Faculty of Medicine, Coimbra University, Coimbra, Portugal, Gonçalves MJ ,   Rheumatology Department, Hospital de Egas Moniz, Unidade Local de Saúde de Lisboa Ocidental, Lisboa, Portugal; CEDOC, NOVA Medical School, Universidade Nova de Lisboa, Lisboa, Portugal, Gomes Guerra M ,   Rheumatology Department, Unidade Local de Saúde da Cova da Beira, Covilhã, Portugal, Ferreira RM ,   Rheumatology Department, Unidade Local de Saúde de São João, Porto, Portugal; 9 Center for Health Technology and Services Research (CINTESIS), Faculty of Medicine, University of Porto, Porto, Portugal, Duarte-Fernandes R ,   Leeds Institute of Rheumatic and Musculoskeletal Medicine, University of Leeds, Leeds, United Kingdom, Barreira S ,   Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal; Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina da Universidade de Lisboa, Lisboa, Portugal, Silvestre-Teixeira C ,   Rheumatology Department, Unidade Local de Saúde do Algarve, Tomás AL ,   Liga Portuguesa Contra as Doenças Reumáticas, Núcleo de Esclerodermia, Romão VC ,   Rheumatology Department, Unidade Local de Saúde Santa Maria, Centro Académico de Medicina de Lisboa, Lisboa, Portugal; Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina da Universidade de Lisboa, Lisboa, Portugal, Cordeiro A ,   Rheumatology Department, Unidade Local de Saúde Almada- Seixal - Hospital Garcia de Orta, Almada, Portugal,   Portuguese Society of Rheumatology/GEDRESIS collaborators
Published: June 2024

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, 2 surgeons (vascular and plastic), 2 nurses, and 1 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.