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Immunoglobulin G4 Related-Disease: A Rare Presentation With Secondary Hypereosinophilic Syndrome and Eosinophilic Ascites

2021-03Journal article Open access
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dc.contributor.authorSerpa Pinto, Luísa
dc.contributor.authorLamas, Nuno Jorge
dc.contributor.authorCampar, Ana
dc.contributor.authorFerreira, Alvaro
dc.contributor.authorCruz, Ana Rita
dc.date.accessioned2023-10-30T12:47:46Z
dc.date.available2023-10-30T12:47:46Z
dc.date.issued2021-03
dc.description.abstractImmunoglobulin G4 related-disease (IgG4-RD) is a multisystemic immune-mediated fibroinflammatory disease, with a strong predilection for salivary and lacrimal glands, pancreas, biliary tree, lungs, kidneys, aorta, and retroperitoneum. In the case of pancreatic involvement, it manifests as autoimmune pancreatitis. Patients with IgG4-RD usually have mild to moderate eosinophilia in the peripheral blood, however, they may present a secondary hypereosinophilic syndrome (HES). Although there are cases described with severe eosinophilia (> 5,000/µL), the 2019 American College of Rheumatology/European League Against Rheumatism classification criteria for IgG4-RD considers peripheral eosinophilia above 3,000/µL as an exclusion criterion, although stating that assessment for the presence of exclusion criteria should be individualized depending on a patient's clinical scenario. Here, we describe the clinical case of a 30-year-old woman who presented with chronic diarrhea and abdominal pain. The diagnostic workup revealed eosinophilic ascites, severe peripheral blood eosinophilia (> 5,000/µL), IgG4 elevation (> 2 × upper normal limit), and also diffuse swelling in the body and pancreatic tail (computed tomography (CT) scan). There was a prompt response to corticosteroid therapy with clinical resolution and continued remission under therapy. The patient was diagnosed with IgG4-RD with secondary HES, explaining the gastrointestinal tract and peritoneum damage in the form of enterocolitis and ascites. Exclusion of alternative diagnosis was made.pt_PT
dc.description.versioninfo:eu-repo/semantics/publishedVersionpt_PT
dc.identifier.citationSerpa Pinto L, Lamas NJ, Campar A, Ferreira A, Cruz AR. Immunoglobulin G4 Related-Disease: A Rare Presentation With Secondary Hypereosinophilic Syndrome and Eosinophilic Ascites. J Med Cases. 2021;12(3):107-111. doi:10.14740/jmc3634pt_PT
dc.identifier.doi10.14740/jmc3634pt_PT
dc.identifier.issn1923-4155
dc.identifier.issn1923-4163
dc.identifier.urihttp://hdl.handle.net/10400.16/2857
dc.language.isoengpt_PT
dc.peerreviewedyespt_PT
dc.publisherElmer Presspt_PT
dc.relation.publisherversionhttps://www.journalmc.org/index.php/JMC/article/view/3634pt_PT
dc.rights.urihttp://creativecommons.org/licenses/by/4.0/pt_PT
dc.subjectAutoimmune pancreatitispt_PT
dc.subjectEosinophilic ascitespt_PT
dc.subjectHypereosinophilic syndromept_PT
dc.subjectIgG4 related-diseasept_PT
dc.subjectSevere eosinophiliapt_PT
dc.titleImmunoglobulin G4 Related-Disease: A Rare Presentation With Secondary Hypereosinophilic Syndrome and Eosinophilic Ascitespt_PT
dc.typejournal article
dspace.entity.typePublication
oaire.citation.conferencePlaceCanadapt_PT
oaire.citation.endPage111pt_PT
oaire.citation.issue3pt_PT
oaire.citation.startPage107pt_PT
oaire.citation.titleJournal of Medical Casespt_PT
oaire.citation.volume12pt_PT
person.familyNameSerpa Pinto
person.familyNameLamas
person.familyNameCampar
person.givenNameLuísa
person.givenNameNuno Jorge
person.givenNameAna
person.identifier1464098
person.identifier.ciencia-idEC14-1A2D-C542
person.identifier.ciencia-idB114-D73D-99FF
person.identifier.orcid0000-0001-5115-4905
person.identifier.orcid0000-0002-4648-136X
person.identifier.orcid0000-0002-3951-8961
person.identifier.ridT-6210-2018
person.identifier.scopus-author-id23100224900
rcaap.rightsopenAccesspt_PT
rcaap.typearticlept_PT
relation.isAuthorOfPublication9653846d-d3c7-4760-b306-0723c927ab22
relation.isAuthorOfPublication89899db7-a439-43c0-94bc-c738cee5324b
relation.isAuthorOfPublication1e019293-8ead-400e-a584-9d14eaa2bc4c
relation.isAuthorOfPublication.latestForDiscovery9653846d-d3c7-4760-b306-0723c927ab22

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