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Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis

2023-04Journal article Open access
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dc.contributor.authorSilva, Ana Bento da
dc.contributor.authorLourenço, Maria Helena
dc.contributor.authorCunha-Branco, Jaime
dc.contributor.authorGonçalves, Maria João
dc.date.accessioned2023-04-21T10:27:11Z
dc.date.embargo2025-04-21
dc.date.issued2023-04
dc.description.abstractA woman with systemic sclerosis presents with a severe and rapidly progressive form of gastrointestinal involvement, mainly marked by recurrent refractory episodes of pseudo-obstruction, culminating in severe malnutrition and dependence on parenteral nutrition. The impact on her quality of life was extremely significant. As a last resort, she started intravenous immunoglobulin with progressive improvement of her symptoms, allowing for the reinstitution of oral diet and removal of parenteral nutrition. After more than 1 year, she maintains clinical stability. Systemic sclerosis has a heterogeneous phenotype, but gastrointestinal involvement is one of the most frequent. Severe manifestations are rare but can lead to severe malnutrition and are associated with high morbidity and mortality rates. Their management is challenging, as the available treatments are still very limited. A better understanding of its pathophysiology, which seems to be unique, is essential to provide more effective treatments and improve quality of life.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationSilva AB, Lourenço MH, Cunha-Branco J, Gonçalves MJ. Particularly severe form of refractory gastrointestinal involvement in systemic sclerosis. BMJ Case Rep. 2023;16(4):e254537.pt_PT
dc.identifier.doi10.1136/bcr-2022-254537pt_PT
dc.identifier.urihttp://hdl.handle.net/10400.21/15925
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherBMJpt_PT
dc.relation.publisherversionhttps://casereports.bmj.com/content/16/4/e254537.longpt_PT
dc.rights.urihttp://creativecommons.org/licenses/by-nc-nd/4.0/pt_PT
dc.subjectConnective tissue diseasept_PT
dc.subjectMalabsorptionpt_PT
dc.subjectMalnutritionpt_PT
dc.subjectParenteral feedingpt_PT
dc.subjectEnteral feedingpt_PT
dc.subjectQuality of lifept_PT
dc.titleParticularly severe form of refractory gastrointestinal involvement in systemic sclerosispt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.issue4pt_PT
oaire.citation.startPagee254537pt_PT
oaire.citation.titleBMJ Case Reportspt_PT
oaire.citation.volume16pt_PT
rcaap.rightsembargoedAccesspt_PT
rcaap.typearticlept_PT

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