%0 Journal Article
%T Portuguese Recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases.
%A Santiago T
%A Duarte AC
%A Sepriano A
%A Castro A
%A Rosa B
%A Resende C
%A Oliveira D
%A Dourado E
%A Costa E
%A Cunha-Santos F
%A Terroso G
%A Boleto G
%A Silva I
%A Barbosa L
%A Silva J
%A Sousa Neves J
%A Salvador MJ
%A Gonçalves MJ
%A Gomes Guerra M
%A Miriam Ferreira R
%A Duarte-Fernandes R
%A Barreira S
%A Silvestre Teixeira V
%A Tomás AL
%A Romão V
%A Cordeiro A
%J ARP Rheumatol
%V 3
%N 2
%D 2024 Apr-Jun
%M 38956991
暂无%X <B>OBJECTIVE: </B>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).<BR><B>METHODS: </B>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.<BR><B>RESULTS: </B>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.<BR><B>CONCLUSIONS: </B>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.