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Open questions on the management of targeted therapies for the treatment of systemic sclerosis-interstitial lung disease: results of a EUSTAR survey based on a systemic literature review

BACKGROUND: The results of randomized controlled (RCT) and retrospective studies have expanded the armamentarium of drugs for systemic sclerosis (SSc) – interstitial lung disease (ILD) treatment. The correct positioning of these drugs is not yet clarified. OBJECTIVES: Systemic literature review (SLR...

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Autores principales: Campochiaro, Corrado, Lazzaroni, Maria Grazia, Bruni, Cosimo, Zanatta, Elisabetta, De Luca, Giacomo, Matucci-Cerinic, Marco
Formato: Online Artículo Texto
Lenguaje:English
Publicado: SAGE Publications 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9425887/
https://www.ncbi.nlm.nih.gov/pubmed/36051631
http://dx.doi.org/10.1177/1759720X221116408
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author Campochiaro, Corrado
Lazzaroni, Maria Grazia
Bruni, Cosimo
Zanatta, Elisabetta
De Luca, Giacomo
Matucci-Cerinic, Marco
author_facet Campochiaro, Corrado
Lazzaroni, Maria Grazia
Bruni, Cosimo
Zanatta, Elisabetta
De Luca, Giacomo
Matucci-Cerinic, Marco
author_sort Campochiaro, Corrado
collection PubMed
description BACKGROUND: The results of randomized controlled (RCT) and retrospective studies have expanded the armamentarium of drugs for systemic sclerosis (SSc) – interstitial lung disease (ILD) treatment. The correct positioning of these drugs is not yet clarified. OBJECTIVES: Systemic literature review (SLR) on rituximab (RTX), tocilizumab (TCZ), nintedanib and abatacept (ABT) for the treatment of SSc-ILD. The results of the SLR were used to create a dedicated survey. DESIGN: The study was performed as a systematic review. DATA SOURCES AND METHODS: the SLR was performed using the following terms: “(systemic sclerosis OR scleroderma) AND (interstitial lung disease OR lung fibrosis OR pulmonary fibrosis) AND (rituximab OR tocilizumab OR abatacept OR nintedanib)”. The results of the SLR were integrated in a survey including 8 domains. These were sent to all EUSTAR members and to the participants of the 2020 Scleroderma World Congress. RESULTS: 41 studies (34 on RTX, 5 on TCZ, 2 on ABT, and 1 on nintedanib) were identified. RCTs supported the use of TCZ and nintedanib, while retrospective studies supported the use of RTX for SSc-ILD. No clear data were obtained about ABT. The survey showed that RTX is the most available option (96%) whereas the most frequent reason for targeted therapy introduction is lung progression while on csDMARDs (86% RTX, 59% TCZ and 63% nintedanib). Combination therapy was the most frequently mentioned therapeutic scheme for nintedanib (75%) and RTX (63%). Physicians’ perception of safety was similar for all drugs, while drug efficacy was the same for RTX and nintedanib, followed by TCZ (4.8 ± 2). The most frequently raised concerns pertained to efficacy, safety and combination regimens. CONCLUSION: Our SLR supports the use of RTX, TCZ and nintedanib for SSc-ILD patients and underlines the need for more data about upfront combination versus monotherapy. It also highlighted the need to identify predictors supporting drug choice according to both pulmonary and extra-pulmonary manifestations.
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spelling pubmed-94258872022-08-31 Open questions on the management of targeted therapies for the treatment of systemic sclerosis-interstitial lung disease: results of a EUSTAR survey based on a systemic literature review Campochiaro, Corrado Lazzaroni, Maria Grazia Bruni, Cosimo Zanatta, Elisabetta De Luca, Giacomo Matucci-Cerinic, Marco Ther Adv Musculoskelet Dis Interstitial Lung Disease in Autoimmune Rheumatic Disorders BACKGROUND: The results of randomized controlled (RCT) and retrospective studies have expanded the armamentarium of drugs for systemic sclerosis (SSc) – interstitial lung disease (ILD) treatment. The correct positioning of these drugs is not yet clarified. OBJECTIVES: Systemic literature review (SLR) on rituximab (RTX), tocilizumab (TCZ), nintedanib and abatacept (ABT) for the treatment of SSc-ILD. The results of the SLR were used to create a dedicated survey. DESIGN: The study was performed as a systematic review. DATA SOURCES AND METHODS: the SLR was performed using the following terms: “(systemic sclerosis OR scleroderma) AND (interstitial lung disease OR lung fibrosis OR pulmonary fibrosis) AND (rituximab OR tocilizumab OR abatacept OR nintedanib)”. The results of the SLR were integrated in a survey including 8 domains. These were sent to all EUSTAR members and to the participants of the 2020 Scleroderma World Congress. RESULTS: 41 studies (34 on RTX, 5 on TCZ, 2 on ABT, and 1 on nintedanib) were identified. RCTs supported the use of TCZ and nintedanib, while retrospective studies supported the use of RTX for SSc-ILD. No clear data were obtained about ABT. The survey showed that RTX is the most available option (96%) whereas the most frequent reason for targeted therapy introduction is lung progression while on csDMARDs (86% RTX, 59% TCZ and 63% nintedanib). Combination therapy was the most frequently mentioned therapeutic scheme for nintedanib (75%) and RTX (63%). Physicians’ perception of safety was similar for all drugs, while drug efficacy was the same for RTX and nintedanib, followed by TCZ (4.8 ± 2). The most frequently raised concerns pertained to efficacy, safety and combination regimens. CONCLUSION: Our SLR supports the use of RTX, TCZ and nintedanib for SSc-ILD patients and underlines the need for more data about upfront combination versus monotherapy. It also highlighted the need to identify predictors supporting drug choice according to both pulmonary and extra-pulmonary manifestations. SAGE Publications 2022-08-22 /pmc/articles/PMC9425887/ /pubmed/36051631 http://dx.doi.org/10.1177/1759720X221116408 Text en © The Author(s), 2022 https://creativecommons.org/licenses/by-nc/4.0/This article is distributed under the terms of the Creative Commons Attribution-NonCommercial 4.0 License (https://creativecommons.org/licenses/by-nc/4.0/) which permits non-commercial use, reproduction and distribution of the work without further permission provided the original work is attributed as specified on the SAGE and Open Access page (https://us.sagepub.com/en-us/nam/open-access-at-sage).
spellingShingle Interstitial Lung Disease in Autoimmune Rheumatic Disorders
Campochiaro, Corrado
Lazzaroni, Maria Grazia
Bruni, Cosimo
Zanatta, Elisabetta
De Luca, Giacomo
Matucci-Cerinic, Marco
Open questions on the management of targeted therapies for the treatment of systemic sclerosis-interstitial lung disease: results of a EUSTAR survey based on a systemic literature review
title Open questions on the management of targeted therapies for the treatment of systemic sclerosis-interstitial lung disease: results of a EUSTAR survey based on a systemic literature review
title_full Open questions on the management of targeted therapies for the treatment of systemic sclerosis-interstitial lung disease: results of a EUSTAR survey based on a systemic literature review
title_fullStr Open questions on the management of targeted therapies for the treatment of systemic sclerosis-interstitial lung disease: results of a EUSTAR survey based on a systemic literature review
title_full_unstemmed Open questions on the management of targeted therapies for the treatment of systemic sclerosis-interstitial lung disease: results of a EUSTAR survey based on a systemic literature review
title_short Open questions on the management of targeted therapies for the treatment of systemic sclerosis-interstitial lung disease: results of a EUSTAR survey based on a systemic literature review
title_sort open questions on the management of targeted therapies for the treatment of systemic sclerosis-interstitial lung disease: results of a eustar survey based on a systemic literature review
topic Interstitial Lung Disease in Autoimmune Rheumatic Disorders
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9425887/
https://www.ncbi.nlm.nih.gov/pubmed/36051631
http://dx.doi.org/10.1177/1759720X221116408
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