ARP Rheumatology
ARP Rheumatology
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Clinical Practice

Acta Reumatológica Portuguesa Apr-Jun 2024; (2) 84-94
Clinical practice, rules or action protocols

Portuguese Recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases

Authors

Santiago T, Duarte AC, Sepriano A, Castro A, Rosa B, Resende C, Oliveira D, Dourado E, Costa E, Cunha-Santos F, Terroso G, Boleto G, Silva I, Barbosa L, Silva J, Neves JS, Salvador MJ, Gonçalves MJ, Guerra MG, Ferreira RM, Duarte-Fernandes R, Barreira S, Teixeira VS, Tomás AL, Romão V, Cordeiro A

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.

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Publication:

2024-06-10

Pubmed:

Cite:

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. Portuguese Recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases. ARP Rheumatology, Vol 3, nº2 2024:84-94. PMID: 38956991
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