2023 Brazilian Society of Rheumatology guidelines for the treatment of systemic sclerosis

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 Rheumatolo...

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Published in:	Advances in rheumatology (London, England) Vol. 64; no. 1; pp. 52 - 16
Main Authors:	Kayser, Cristiane, de Oliveira Delgado, Sandra Maximiano, Zimmermann, Adriana Fontes, Horimoto, Alex Magno Coelho, Del Rio, Ana Paula Toledo, de Souza Müller, Carolina, Camargo, Cintia Zumstein, Lupo, Cristiano Michelini, de Moraes, Daniela Aparecida, Do Rosário E Souza, Eduardo José, Santos, Flávia Patrícia Sena Teixeira, Sekiyama, Juliana Yuri, Lonzetti, Lilian Scussel, de Oliveira Martins, Lucas Victória, Bezerra, Mailze Campos, Bredemeier, Markus, Oliveira, Maria Carolina, da Fonseca Salgado, Maria Cecília, Miossi, Renata, de Araújo Fontenele, Sheila Márcia, Hax, Vanessa, Dantas, Andrea Tavares, Sampaio-Barros, Percival Degrava
Format:	Journal Article
Language:	English
Published:	England Sociedade Brasileira de Reumatologia 10-07-2024 BMC
Subjects:	Antibodies, Monoclonal, Humanized - therapeutic use Antirheumatic Agents - therapeutic use Brazil Disease management Humans Interstitial lung disease Lung Diseases, Interstitial - drug therapy Randomized Controlled Trials as Topic Raynaud Disease - drug therapy Raynaud’s phenomenon RHEUMATOLOGY Rheumatology - standards Rituximab - therapeutic use Scleroderma Scleroderma, Systemic - complications Scleroderma, Systemic - drug therapy Skin Ulcer - etiology Societies, Medical Systemic sclerosis Treatment Brazil Digital ulcer Raynaud’s phenomenon Treatment Systemic sclerosis Disease management Interstitial lung disease Scleroderma Guidelines
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Summary:	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. 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. 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. 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.
Bibliography:	ObjectType-Article-1 SourceType-Scholarly Journals-1 ObjectType-Feature-2 ObjectType-Instructional Material/Guideline-3 content type line 23 ObjectType-Undefined-4
ISSN:	2523-3106 2523-3106
DOI:	10.1186/s42358-024-00392-w