Systematic literature review to inform the Portuguese recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases.

Objective: To perform a systematic literature review (SLR) aimed at evaluating the efficacy and safety of pharmacological and non-pharmacological treatments for Raynaud's phenomenon (RP) and digital ulcers (DU) in patients with systemic sclerosis (SSc) and other connective tissue diseases (CTD), in...

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Publicado en:ARP Rheumatology Vol. 3; no. 2; pp. 128 - 207
Autores principales: Costa, E., Cunha-Santos, F., Dourado, E., Oliveira, D., Falzon, L., Romão, V. C., Duarte, A. C., Cordeiro, A., Santiago, T., Sepriano, A.
Formato: Artículo
Publicado: Sociedade Portuguesa de Reumatologia Apr-Jun2024
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Acceso en línea:Ver este registro en EBSCOhost
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      dt: Apr-Jun2024
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      pub: Sociedade Portuguesa de Reumatologia
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        atl: Systematic literature review to inform the Portuguese recommendations for the management of Raynaud's phenomenon and digital ulcers in systemic sclerosis and other connective tissue diseases.
      aug:
        au:
          Costa, E.
          Cunha-Santos, F.
          Dourado, E.
          Oliveira, D.
          Falzon, L.
          Romão, V. C.
          Duarte, A. C.
          Cordeiro, A.
          Santiago, T.
          Sepriano, A.
        affil:
          Rheumatology Department, Hospital de Braga, Braga, Portugal
          Rheumatology Department, Unidade Local de Saúde da Guarda, Guarda, Portugal
          Rheumatology Department, Centro Hospitalar do Baixo Vouga, Aveiro, Portugal
          Aveiro Rheumatology Research Centre, Egas Moniz Health Alliance
          Rheumatology Department, Centro Hospitalar Universitário de São João, Porto, Portugal
          Center for Health Technology and Services Research (CINTESIS), Faculdade de Medicina da Universidade do Porto, Porto, Portugal
          Health Economics and Decision Science, School of Medicine and Population Health, University of Sheffield, Sheffield, United Kingdom
          Rheumatology Department, Centro Hospitalar Universitário Lisboa Norte, Centro Académico de Medicina de Lisboa, Lisbon, Portugal
          Rheumatology Research Unit, Instituto de Medicina Molecular, Faculdade de Medicina, Universidade de Lisboa, Centro Académico de Medicina de Lisboa, Lisbon, Portugal
          Rheumatology Department, Hospital Garcia de Orta, Almada, Portugal
          Rheumatology Department, Centro Hospitalar e Universitário de Coimbra, Coimbra, Portugal
          Faculty of Medicine, University of Coimbra, Coimbra, Portugal
          NOVA Medical School, Universidade Nova de Lisboa, Lisbon, Portuga
          Rheumatology Department, Centro Hospitalar de Lisboa Ocidental, Lisbon, Portugal
      su:
        Raynaud's disease
        Systemic scleroderma
        Connective tissue diseases
        Phosphodiesterase-5 inhibitors
        Phosphodiesterase inhibitors
        Randomized controlled trials
        Calcium antagonists
      sug:
        subj:
          Raynaud's disease
          Systemic scleroderma
          Connective tissue diseases
          Phosphodiesterase-5 inhibitors
          Phosphodiesterase inhibitors
          Randomized controlled trials
          Calcium antagonists
      keyword:
        Connective Tissue Diseases
        Digital Ulcers
        Raynaud Phenomenon
        Scleroderma and related disorders
        Systematic Literature Review
      ab: Objective: To perform a systematic literature review (SLR) aimed at evaluating the efficacy and safety of pharmacological and non-pharmacological treatments for Raynaud's phenomenon (RP) and digital ulcers (DU) in patients with systemic sclerosis (SSc) and other connective tissue diseases (CTD), in order to inform the Portuguese recommendations for managing RP and DU in these patients. Methods: A SLR was conducted until May 2022 to identify studies assessing the efficacy and safety of pharmacological and non-pharmacological interventions for RP and DU in SSc and other CTD. Eligible study designs included randomized controlled trials (RCTs), controlled clinical trials, and their extensions for assessing efficacy and safety of interventions. Observational studies with a comparator were included for evaluating the efficacy and safety of non-pharmacological interventions and safety of pharmacological interventions. The risk of bias of each study was assessed using standard tools. Results: Out of 71 publications meeting the inclusion criteria, 59 evaluated pharmacological and 12 nonpharmacological interventions. We found moderate quality evidence supporting the efficacy of calcium channel blockers, phosphodiesterase-5 inhibitors, and intravenous prostacyclin analogues in reducing RP frequency, severity, and duration. Intravenous iloprost had a small to moderate effect size in improving DU healing. Phosphodiesterase-5 inhibitors were effective in reducing total DU count, new DU occurrence, and enhancing DU healing. Bosentan effectively prevented new DU in SSc patients. No new safety concerns were associated with these treatments. The studies on non-pharmacological interventions were, in general, of low quality, and had a small sample size. Warming measures decreased frequency and duration of RP attacks; laser therapy improved RP-related outcomes; local oxygen-ozone therapy improved RP outcomes as an add-on therapy; bone marrow mononuclear cell implantation improved DU-associated pain; periarterial sympathectomy and vascular bypass reduced DU number and finger amputation risk. Conclusion: The available evidence supports the efficacy and safety of pharmacological interventions, namely nifedipine, sildenafil, iloprost, and bosentan in treating RP and DU in patients with SSc and other CTD. Scarce and low-quality evidence does support the use of some non-pharmacological interventions but with only a modest effect size. This SLR underscores the limited availability of high-quality evidence for determining the optimal treatment of RP and/or DUs, emphasising the need for further studies to evaluate efficacy and safety aspects.
      pubtype: Academic Journal
      doctype: Article
      src: R
    language: English
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      custom: Copyright of ARP Rheumatology is the property of Sociedade Portuguesa de Reumatologia and its content may not be copied or emailed to multiple sites without the copyright holder's express written permission. Additionally, content may not be used with any artificial intelligence tools or machine learning technologies. However, users may print, download, or email articles for individual use.
      item: ARP Rheumatology
      holder: Sociedade Portuguesa de Reumatologia
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          year: 2024
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