%0 Journal Article
%T 2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis.
%A Kayser C
%A de Oliveira Delgado SM
%A Zimmermann AF
%A Horimoto AMC
%A Del Rio APT
%A de Souza Müller C
%A Camargo CZ
%A Lupo CM
%A de Moraes DA
%A Do Rosário E Souza EJ
%A Santos FPST
%A Sekiyama JY
%A Lonzetti LS
%A de Oliveira Martins LV
%A Bezerra MC
%A Bredemeier M
%A Oliveira MC
%A da Fonseca Salgado MC
%A Miossi R
%A de Araújo Fontenele SM
%A Hax V
%A Dantas AT
%A Sampaio-Barros PD
%J Adv Rheumatol
%V 64
%N 1
%D 2024 Jul 10
%M 38987832
%F 3.01
%R 10.1186/s42358-024-00392-w
%X BACKGROUND: Systemic sclerosis (SSc) is a rare chronic autoimmune disease with heterogeneous manifestations. In the last decade, several clinical trials have been conducted to evaluate new treatment options for SSc. The purpose of this work is to update the recommendations of the Brazilian Society of Rheumatology in light of the new evidence available for the pharmacological management of SSc.
METHODS: A systematic review including randomized clinical trials (RCTs) for predefined questions that were elaborated according to the Patient/Population, Intervention, Comparison, and Outcomes (PICO) strategy was conducted. The rating of the available evidence was performed according to the Grading of Recommendations Assessment, Development and Evaluation (GRADE) methodology. To become a recommendation, at least 75% agreement of the voting panel was needed.
RESULTS: Six recommendations were elaborated regarding the pharmacological treatment of Raynaud's phenomenon, the treatment (healing) and prevention of digital ulcers, skin involvement, interstitial lung disease (ILD) and gastrointestinal involvement in SSc patients based on results available from RCTs. New drugs, such as rituximab, were included as therapeutic options for skin involvement, and rituximab, tocilizumab and nintedanib were included as therapeutic options for ILD. Recommendations for the pharmacological treatment of scleroderma renal crisis and musculoskeletal involvement were elaborated based on the expert opinion of the voting panel, as no placebo-controlled RCTs were found.
CONCLUSIONS: These guidelines updated and incorporated new treatment options for the management of SSc based on evidence from the literature and expert opinion regarding SSc, providing support for decision-making in clinical practice.