Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome.
Identifieur interne : 000776 ( Ncbi/Curation ); précédent : 000775; suivant : 000777Antifibrotics in interstitial lung disease related to connective tissue diseases - a paradigm shift in treatment and outcome.
Auteurs : Ana Catarina Duarte ; Filipe Vinagre ; Jorge Soares ; Ana CordeiroSource :
- Acta reumatologica portuguesa [ 0303-464X ]
Descripteurs français
- KwdFr :
- Acide mycophénolique (usage thérapeutique), Adulte d'âge moyen, Azathioprine (usage thérapeutique), Cyclophosphamide (usage thérapeutique), Femelle, Humains, Hydroxychloroquine (usage thérapeutique), Indoles (usage thérapeutique), Infliximab (usage thérapeutique), Pneumopathies interstitielles (traitement médicamenteux), Pneumopathies interstitielles (étiologie), Prednisolone (usage thérapeutique), Pyridones (usage thérapeutique), Résultat thérapeutique, Sclérodermie systémique (), Substitution de médicament, Sujet âgé, Syndrome de Gougerot-Sjögren ().
- MESH :
- traitement médicamenteux : Pneumopathies interstitielles.
- usage thérapeutique : Acide mycophénolique, Azathioprine, Cyclophosphamide, Hydroxychloroquine, Indoles, Infliximab, Prednisolone, Pyridones.
- étiologie : Pneumopathies interstitielles.
- Adulte d'âge moyen, Femelle, Humains, Résultat thérapeutique, Sclérodermie systémique, Substitution de médicament, Sujet âgé, Syndrome de Gougerot-Sjögren.
English descriptors
- KwdEn :
- Aged, Azathioprine (therapeutic use), Cyclophosphamide (therapeutic use), Drug Substitution, Female, Humans, Hydroxychloroquine (therapeutic use), Indoles (therapeutic use), Infliximab (therapeutic use), Lung Diseases, Interstitial (drug therapy), Lung Diseases, Interstitial (etiology), Middle Aged, Mycophenolic Acid (therapeutic use), Prednisolone (therapeutic use), Pyridones (therapeutic use), Scleroderma, Systemic (complications), Sjogren's Syndrome (complications), Treatment Outcome.
- MESH :
- chemical , therapeutic use : Azathioprine, Cyclophosphamide, Hydroxychloroquine, Indoles, Infliximab, Mycophenolic Acid, Prednisolone, Pyridones.
- complications : Scleroderma, Systemic, Sjogren's Syndrome.
- drug therapy : Lung Diseases, Interstitial.
- etiology : Lung Diseases, Interstitial.
- Aged, Drug Substitution, Female, Humans, Middle Aged, Treatment Outcome.
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.
PubMed: 31280278
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Ana Catarina Duarte<affiliation><nlm:affiliation>Hospital Garcia de Orta.</nlm:affiliation>
<wicri:noCountry code="no comma">Hospital Garcia de Orta.</wicri:noCountry>
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Le document en format XML
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<term>Sclérodermie systémique</term>
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<front><div type="abstract" xml:lang="en">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.</div>
</front>
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