ARP Rheumatology
ARP Rheumatology
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Article

ARP Rheumatology
Letter

Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome

Authors

Duarte AC, Vinagre F, Soares J, Cordeiro A

Abstract

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.

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Publication:

2019-05-18

Pubmed:

Cite:

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. ARP, Vol 44, nº2 2019:161-162. PMID: 31280278
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