Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome
Interstitial lung disease (ILD) is one of the major causes of morbidity and mortality in patients with connective tissue disease (CTD) and the treatments available until nowadays are in most cases unable to halt disease progression. CTD-ILD pathogenesis includes an initial inflammatory phase, fol...
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Sociedade Portuguesa de Reumatologia
2019-04-01
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doaj-e1a7567d3c8346ada61363d7ce4e3ee52020-11-25T01:56:14ZengSociedade Portuguesa de ReumatologiaActa Reumatológica Portuguesa0303-464X2019-04-0120192161162190070Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcomeAna Catarina Duarte0Filipe Vinagre1Jorge Soares2Ana Cordeiro3Hospital Garcia de OrtaHospital Garcia de OrtaHospital Garcia de OrtaHospital Garcia de OrtaInterstitial lung disease (ILD) is one of the major causes of morbidity and mortality in patients with connective tissue disease (CTD) and the treatments available until nowadays are in most cases unable to halt disease progression. CTD-ILD pathogenesis includes an initial inflammatory phase, followed by a fibrotic phase, in which extracellular matrix proteins are produced and fibrotic scaring tissue within the lung develops. Steroids and immunosuppressants are the weapons we currently have to treat CTD-ILD. However, mortality rates remain high and identification of new therapeutic targets is crucial. Antifibrotic drugs, which include nintedanib and pirfenidone, have been approved for the treatment of idiopathic pulmonary fibrosis (IPF) and due to similar pathogenesis between IPF and CTD-ILD, their use seems attractive in patients with CTD-IL. We report 3 cases of patients with different CTDs, with predominantly fibrotic changes in high resolution computed tomography that progressed despite immunosuppression, and who have attained disease stability after introduction of antifibrotic drugs.http://www.actareumatologica.com/files/article/1255_antifibrotics_in_interstitial_lung_disease_related_to_connective_tissue_diseases_a_paradigm_shift_in_treatment_and_outcome__file.pdfantifibrotic drugsconnective tissue diseasepirfenidoneinterstitial lung diseasenintedanib |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Ana Catarina Duarte Filipe Vinagre Jorge Soares Ana Cordeiro |
spellingShingle |
Ana Catarina Duarte Filipe Vinagre Jorge Soares Ana Cordeiro Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome Acta Reumatológica Portuguesa antifibrotic drugs connective tissue disease pirfenidone interstitial lung disease nintedanib |
author_facet |
Ana Catarina Duarte Filipe Vinagre Jorge Soares Ana Cordeiro |
author_sort |
Ana Catarina Duarte |
title |
Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome |
title_short |
Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome |
title_full |
Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome |
title_fullStr |
Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome |
title_full_unstemmed |
Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome |
title_sort |
antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome |
publisher |
Sociedade Portuguesa de Reumatologia |
series |
Acta Reumatológica Portuguesa |
issn |
0303-464X |
publishDate |
2019-04-01 |
description |
Interstitial lung disease (ILD) is one of the major causes of morbidity and mortality in patients with connective tissue disease (CTD) and the treatments available until nowadays are in most cases unable to halt disease progression.
CTD-ILD pathogenesis includes an initial inflammatory phase, followed by a fibrotic phase, in which extracellular matrix proteins are produced and fibrotic scaring tissue within the lung develops. Steroids and immunosuppressants are the weapons we currently have to treat CTD-ILD. However, mortality rates remain high and identification of new therapeutic targets is crucial. Antifibrotic drugs, which include nintedanib and pirfenidone, have been approved for the treatment of idiopathic pulmonary fibrosis (IPF) and due to similar pathogenesis between IPF and CTD-ILD, their use seems attractive in patients with CTD-IL.
We report 3 cases of patients with different CTDs, with predominantly fibrotic changes in high resolution computed tomography that progressed despite immunosuppression, and who have attained disease stability after introduction of antifibrotic drugs. |
topic |
antifibrotic drugs connective tissue disease pirfenidone interstitial lung disease nintedanib |
url |
http://www.actareumatologica.com/files/article/1255_antifibrotics_in_interstitial_lung_disease_related_to_connective_tissue_diseases_a_paradigm_shift_in_treatment_and_outcome__file.pdf |
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