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Anti-fibrotic nintedanib—a new opportunity for systemic sclerosis patients?

dc.contributor.authorDuarte, Ana Catarina
dc.contributor.authorSantos, Maria José
dc.contributor.authorCordeiro, Ana
dc.date.accessioned2019-12-16T12:50:45Z
dc.date.available2019-12-16T12:50:45Z
dc.date.issued2018-04
dc.description.abstractSystemic sclerosis is a connective tissue disease characterized by progressive skin thickening and a wide spectrum of internal organ involvement. Pathogenesis includes vasculopathy, inflammation, and fibrosis. Although immunosuppressants such as cyclophosphamide and mycophenolate mofetil have shown some benefit in interstitial lung disease management, it is still a major cause of morbi-mortality in these patients. Therefore, there is a current need for new therapies. Here, we report a 65-year-old female patient with limited cutaneous systemic sclerosis, anti-topoisomerase-positive and extensive lung disease. The patient developed progressive lung fibrosis under several immunosuppressants and was started on nintedanib, with clinical and functional stabilization. Nintedanib is a tyrosine-kinase inhibitor that blocks several profibrotic pathways, inhibiting proliferation and migration of fibroblasts and decreasing the synthesis of extracellular matrix proteins. It is approved for idiopathic lung fibrosis and has demonstrated good results in inhibiting migration and proliferation of systemic sclerosis dermal fibroblasts, constituting a promising agent for systemic sclerosis-associated lung fibrosis.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationDuarte, A.C., Santos, M.J. & Cordeiro, A. Clin Rheumatol (2018) 37: 1123. https://doi.org/10.1007/s10067-017-3867-3pt_PT
dc.identifier.doi10.1007/s10067-017-3867-3pt_PT
dc.identifier.issn0770-3198
dc.identifier.issn1434-9949
dc.identifier.urihttp://hdl.handle.net/10400.26/30503
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherSpringerpt_PT
dc.relation.publisherversionhttps://doi.org/10.1007/s10067-017-3867-3pt_PT
dc.subjectAnti-fibrotic therapypt_PT
dc.subjectInterstitial lung diseasept_PT
dc.subjectNintedanibpt_PT
dc.subjectSystemic sclerosispt_PT
dc.titleAnti-fibrotic nintedanib—a new opportunity for systemic sclerosis patients?pt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.endPage1127pt_PT
oaire.citation.startPage1123pt_PT
oaire.citation.titleClinical Rheumatologypt_PT
oaire.citation.volume37(4)pt_PT
rcaap.embargofctPolítica de copyright do editorpt_PT
rcaap.rightsrestrictedAccesspt_PT
rcaap.typearticlept_PT

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