Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies

OBJECTIVE: To compare the efficacy, toxicity and glucocorticoid (GC)-sparing effects of intravenous cyclophosphamide (iv CYC) with other immunosuppressive regimes as the induction treatment for Idiopathic Inflammatory Myopathy-Related Interstitial Lung Disease (IIM-ILD). METHODS: Observational compa...

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Autores: Moreno Torres, Víctor, Martín Iglesias, Daniel, Vivero, Florencia, González Echavarri, Cristina, García Moyano, Marta, Enghelmayer, Juan Ignacio, Malfante, Pablo, Gaser, Adrián, Ruiz Irastorza, Guillermo, EPIMAR cohort investigators
Tipo de recurso: artículo
Fecha de publicación:2023
País:España
Institución:Universidad del País Vasco
Repositorio:Addi. Archivo Digital para la Docencia y la Investigación
OAI Identifier:oai:addi.ehu.eus:10810/59914
Acceso en línea:http://hdl.handle.net/10810/59914
Access Level:acceso abierto
Palabra clave:idiopathic inflammatory myopathies
interstitial lung disease
cyclophosphamide
glucocorticoids
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spelling Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathiesMoreno Torres, VíctorMartín Iglesias, DanielVivero, FlorenciaGonzález Echavarri, CristinaGarcía Moyano, MartaEnghelmayer, Juan IgnacioMalfante, PabloGaser, AdriánRuiz Irastorza, GuillermoEPIMAR cohort investigatorsidiopathic inflammatory myopathiesinterstitial lung diseasecyclophosphamideglucocorticoidsOBJECTIVE: To compare the efficacy, toxicity and glucocorticoid (GC)-sparing effects of intravenous cyclophosphamide (iv CYC) with other immunosuppressive regimes as the induction treatment for Idiopathic Inflammatory Myopathy-Related Interstitial Lung Disease (IIM-ILD). METHODS: Observational comparative study of patients with IIM-ILD from the EPIMAR and Cruces cohorts. The main efficacy outcome was a 6 to 12-month improvement >10% in the forced vital capacity (FVC) from baseline. RESULTS: Overall, 47 patients were included: 22 (47%) in the CYC group and 25 (53%) in the non-CYC group (32% azathioprine, 28% GC alone, 20% mycophenolate, 16% calcineurin-inhibitors and methotrexate and 4% rituximab). 81% patients were female with a mean age of 50.4 years. FVC improvement was achieved by 64% patients in the CYC group vs. 32% in the non-CYC group (p=0.03). In the logistic regression model, CYC was identified as the only independent predictor of FVC improvement (OR=3.97, 95% CI 1.07-14.75). Patients in the CYC group received more methyl-prednisolone pulses (MP) (59% vs. 28% in the non-CYC group, p=0.03), less initial GCs doses >30mg/d (19% vs. 77%, p=0.001) and lower 6-month average doses of prednisone (11mg/d vs. 31.1mg/d, p=0.001). CONCLUSION: iv CYC showed better functional outcomes than other immunosuppressants in IIM-ILD. The additional use of MP is likely to potentiate the effects of CYC and allows lowering prednisone doses. Therefore, CYC in combination with MP could be considered as the first line induction therapy in IIM-ILD, without limiting its use to rapidly progressive, life-threatening or refractory disease.Dr. Ruiz-Irastorza was supported by the Department of Education of the Basque Government, research grant IT 1512–22.Elsevier202320232023info:eu-repo/semantics/articleapplication/pdfhttp://hdl.handle.net/10810/59914reponame:Addi. Archivo Digital para la Docencia y la Investigacióninstname:Universidad del País VascoIngléshttps://www.sciencedirect.com/science/article/pii/S0049017223000045?via%3Dihubinfo:eu-repo/semantics/openAccesshttp://creativecommons.org/licenses/by/3.0/es/© 2023 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/)Atribución 3.0 Españaoai:addi.ehu.eus:10810/599142026-06-18T09:23:17Z
dc.title.none.fl_str_mv Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies
title Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies
spellingShingle Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies
Moreno Torres, Víctor
idiopathic inflammatory myopathies
interstitial lung disease
cyclophosphamide
glucocorticoids
title_short Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies
title_full Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies
title_fullStr Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies
title_full_unstemmed Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies
title_sort Intravenous cyclophosphamide improves functional outcomes in interstitial lung disease related to idiopathic inflammatory myopathies
dc.creator.none.fl_str_mv Moreno Torres, Víctor
Martín Iglesias, Daniel
Vivero, Florencia
González Echavarri, Cristina
García Moyano, Marta
Enghelmayer, Juan Ignacio
Malfante, Pablo
Gaser, Adrián
Ruiz Irastorza, Guillermo
EPIMAR cohort investigators
author Moreno Torres, Víctor
author_facet Moreno Torres, Víctor
Martín Iglesias, Daniel
Vivero, Florencia
González Echavarri, Cristina
García Moyano, Marta
Enghelmayer, Juan Ignacio
Malfante, Pablo
Gaser, Adrián
Ruiz Irastorza, Guillermo
EPIMAR cohort investigators
author_role author
author2 Martín Iglesias, Daniel
Vivero, Florencia
González Echavarri, Cristina
García Moyano, Marta
Enghelmayer, Juan Ignacio
Malfante, Pablo
Gaser, Adrián
Ruiz Irastorza, Guillermo
EPIMAR cohort investigators
author2_role author
author
author
author
author
author
author
author
author
dc.subject.none.fl_str_mv idiopathic inflammatory myopathies
interstitial lung disease
cyclophosphamide
glucocorticoids
topic idiopathic inflammatory myopathies
interstitial lung disease
cyclophosphamide
glucocorticoids
description OBJECTIVE: To compare the efficacy, toxicity and glucocorticoid (GC)-sparing effects of intravenous cyclophosphamide (iv CYC) with other immunosuppressive regimes as the induction treatment for Idiopathic Inflammatory Myopathy-Related Interstitial Lung Disease (IIM-ILD). METHODS: Observational comparative study of patients with IIM-ILD from the EPIMAR and Cruces cohorts. The main efficacy outcome was a 6 to 12-month improvement >10% in the forced vital capacity (FVC) from baseline. RESULTS: Overall, 47 patients were included: 22 (47%) in the CYC group and 25 (53%) in the non-CYC group (32% azathioprine, 28% GC alone, 20% mycophenolate, 16% calcineurin-inhibitors and methotrexate and 4% rituximab). 81% patients were female with a mean age of 50.4 years. FVC improvement was achieved by 64% patients in the CYC group vs. 32% in the non-CYC group (p=0.03). In the logistic regression model, CYC was identified as the only independent predictor of FVC improvement (OR=3.97, 95% CI 1.07-14.75). Patients in the CYC group received more methyl-prednisolone pulses (MP) (59% vs. 28% in the non-CYC group, p=0.03), less initial GCs doses >30mg/d (19% vs. 77%, p=0.001) and lower 6-month average doses of prednisone (11mg/d vs. 31.1mg/d, p=0.001). CONCLUSION: iv CYC showed better functional outcomes than other immunosuppressants in IIM-ILD. The additional use of MP is likely to potentiate the effects of CYC and allows lowering prednisone doses. Therefore, CYC in combination with MP could be considered as the first line induction therapy in IIM-ILD, without limiting its use to rapidly progressive, life-threatening or refractory disease.
publishDate 2023
dc.date.none.fl_str_mv 2023
2023
2023
dc.type.none.fl_str_mv info:eu-repo/semantics/article
format article
dc.identifier.none.fl_str_mv http://hdl.handle.net/10810/59914
url http://hdl.handle.net/10810/59914
dc.language.none.fl_str_mv Inglés
language_invalid_str_mv Inglés
dc.relation.none.fl_str_mv https://www.sciencedirect.com/science/article/pii/S0049017223000045?via%3Dihub
dc.rights.none.fl_str_mv info:eu-repo/semantics/openAccess
http://creativecommons.org/licenses/by/3.0/es/
Atribución 3.0 España
eu_rights_str_mv openAccess
rights_invalid_str_mv http://creativecommons.org/licenses/by/3.0/es/
Atribución 3.0 España
dc.format.none.fl_str_mv application/pdf
dc.publisher.none.fl_str_mv Elsevier
publisher.none.fl_str_mv Elsevier
dc.source.none.fl_str_mv reponame:Addi. Archivo Digital para la Docencia y la Investigación
instname:Universidad del País Vasco
instname_str Universidad del País Vasco
reponame_str Addi. Archivo Digital para la Docencia y la Investigación
collection Addi. Archivo Digital para la Docencia y la Investigación
repository.name.fl_str_mv
repository.mail.fl_str_mv
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score 15,300724

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