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

Santiago, T.; Duarte, A. C.; Sepriano, A.; Castro, A.; Rosa, B.; Resende, C.; Oliveira, D.; Dourado, D.; Costa, E.; Santos, F. Cunha; Terroso, G.; Boleto, G.; Silva, I.; Barbosa, L.; Silva, J.; Neves, J. Sousa; Salvador, M. J.; Gonçalves, M. J.; Guerra, M. Gomes; Ferreira, R. M.; Fernandes, R. Duarte; Barreira, S.; Teixeira, C. Silvestre; Tomás, A. L.; Romão, V. C.; Cordeiro, A.

http://hdl.handle.net/10400.1/26494

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Abstract(s)

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.