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

Kayser, Cristiane; Delgado, Sandra Maximiano de Oliveira; Zimmermann, Adriana Fontes; Horimoto, Alex Magno Coelho; Del Rio, Ana Paula Toledo; Müller, Carolina de Souza; Camargo, Cintia Zumstein; Lupo, Cristiano Michelini; Moraes, Daniela Aparecida de; Do Rosário E Souza, Eduardo José; Santos, Flávia Patrícia Sena Teixeira; Sekiyama, Juliana Yuri; Lonzetti, Lilian Scussel; Martins, Lucas Victória de Oliveira; Bezerra, Mailze Campos; Bredemeier, Markus; Oliveira, Maria Carolina; Salgado, Maria Cecília da Fonseca; Miossi, Renata; Fontenele, Sheila Márcia de Araújo; Hax, Vanessa; Dantas, Andrea Tavares; Sampaio-Barros, Percival Degrava

doi:10.1186/s42358-024-00392-w

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Sumário

POSITION ARTICLE AND GUIDELINES • Adv. rheumatol. 64 • 2024 • https://doi.org/10.1186/s42358-024-00392-w copy

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

Authorship SCIMAGO INSTITUTIONS RANKINGS

Abstract

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.

Conclusion

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.

Systemic sclerosis; Scleroderma; Treatment; Disease management; Interstitial lung disease; Raynaud’s phenomenon; Digital ulcer; Guidelines

Strength of the recommendation	Description
Strong recommendation	The desirable effects of intervention clearly outweigh its undesirable effects
Conditional recommendation	The desirable effects probably outweigh the undesirable effects, but appreciable uncertainty exists
Conditional recommendation against	The undesirable effects probably outweigh the desirable effect, but appreciable uncertainty exists
Strong recommendation against	The undesirable effects of an intervention outweigh its desirable effect

Certainty
High	The authors have a lot of confidence that the true effect lies close to the estimated effect
Moderate	The authors are moderately confident that the true effect is probably close to the estimated effect
Low	The confidence in the estimated effect is limited: the true effect may be substantially different from the estimated effect
Very low	The authors have very little confidence in the estimated effect: the true effect is likely to be substantially different from the estimated effect

Clinical question	Recommendation	Strength of recommendation	Quality of evidence	Degree of agreement (%)
What is the evidence for the pharmacological treatment of Raynaud’s phenomenon?	Calcium channel blockers (dihydropyridine-type)	Strong	Very low	87
	PDE-5 inhibitors (sildenafil)	Strong	Moderate	95.7
	Intravenous prostacyclin analogs	Conditional	Very low	91.3
What evidence is available for the treatment of digital ulcers (DUs)?	Phosphodiesterase type 5 inhibitors	Strong	Low	100
	Intravenous prostacyclin analogs	Conditional	Very low	95.7
	Adipose tissue graft	Conditional	Very low	95.7
What evidence is available for preventing recurrence of DUs?	Bosentan	Strong	Moderate	82.6
	Phosphodiesterase type 5 inhibitors	Strong	Low	100
	Intravenous prostacyclin analogs	Conditional	Very low	95.7
What is the evidence for the treatment of skin involvement?	Methotrexate	Strong	Moderate	96
	Mycophenolate	Conditional	Low	83
	Cyclophosphamide	Conditional	Moderate	87
	Rituximab	Conditional	Low	96
	Autologous stem-cell transplantation	Strong	Moderate	78.3
What is the evidence for the treatment of interstitial lung disease?	Cyclophosphamide	Conditional	Low	78
	Mycophenolate	Conditional	Low	91
	Nintedanib	Conditional	Low	87
	Rituximab	Conditional	Low	83
	Tocilizumab	Conditional	Moderate	91
	Autologous stem-cell transplantation	Conditional	Low	96
What therapeutic evidence is available for the treatment of gastrointestinal involvement?	Prucalopride	Conditional	Very low	95.7
What treatments are beneficial in the management of scleroderma renal crisis?	Angiotensin-converting enzyme inhibitors	–	Expert opinion	87
What treatments are beneficial in the management of musculoskeletal manifestations (arthritis and/or myositis)?	Low-dose corticosteroids and methotrexate	–	Expert opinion	78.3

Sociedade Brasileira de Reumatologia Av. Brigadeiro Luís Antônio, 2466, Jardim Paulista, 01402-000 - São Paulo, SP, Tel.: +551132897165 - São Paulo - SP - Brazil
E-mail: rbreumatol@terra.com.br

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[1] *Correspondence: Cristiane Kayser cristiane.kayser@unifesp.br