Publication
Immunoglobulin G4 Related-Disease: A Rare Presentation With Secondary Hypereosinophilic Syndrome and Eosinophilic Ascites
dc.contributor.author | Serpa Pinto, Luísa | |
dc.contributor.author | Lamas, Nuno Jorge | |
dc.contributor.author | Campar, Ana | |
dc.contributor.author | Ferreira, Alvaro | |
dc.contributor.author | Cruz, Ana Rita | |
dc.date.accessioned | 2023-10-30T12:47:46Z | |
dc.date.available | 2023-10-30T12:47:46Z | |
dc.date.issued | 2021-03 | |
dc.description.abstract | Immunoglobulin 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.version | info:eu-repo/semantics/publishedVersion | pt_PT |
dc.identifier.citation | Serpa 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/jmc3634 | pt_PT |
dc.identifier.doi | 10.14740/jmc3634 | pt_PT |
dc.identifier.issn | 1923-4155 | |
dc.identifier.issn | 1923-4163 | |
dc.identifier.uri | http://hdl.handle.net/10400.16/2857 | |
dc.language.iso | eng | pt_PT |
dc.peerreviewed | yes | pt_PT |
dc.publisher | Elmer Press | pt_PT |
dc.relation.publisherversion | https://www.journalmc.org/index.php/JMC/article/view/3634 | pt_PT |
dc.rights.uri | http://creativecommons.org/licenses/by/4.0/ | pt_PT |
dc.subject | Autoimmune pancreatitis | pt_PT |
dc.subject | Eosinophilic ascites | pt_PT |
dc.subject | Hypereosinophilic syndrome | pt_PT |
dc.subject | IgG4 related-disease | pt_PT |
dc.subject | Severe eosinophilia | pt_PT |
dc.title | Immunoglobulin G4 Related-Disease: A Rare Presentation With Secondary Hypereosinophilic Syndrome and Eosinophilic Ascites | pt_PT |
dc.type | journal article | |
dspace.entity.type | Publication | |
oaire.citation.conferencePlace | Canada | pt_PT |
oaire.citation.endPage | 111 | pt_PT |
oaire.citation.issue | 3 | pt_PT |
oaire.citation.startPage | 107 | pt_PT |
oaire.citation.title | Journal of Medical Cases | pt_PT |
oaire.citation.volume | 12 | pt_PT |
person.familyName | Serpa Pinto | |
person.familyName | Lamas | |
person.familyName | Campar | |
person.givenName | Luísa | |
person.givenName | Nuno Jorge | |
person.givenName | Ana | |
person.identifier | 1464098 | |
person.identifier.ciencia-id | EC14-1A2D-C542 | |
person.identifier.ciencia-id | B114-D73D-99FF | |
person.identifier.orcid | 0000-0001-5115-4905 | |
person.identifier.orcid | 0000-0002-4648-136X | |
person.identifier.orcid | 0000-0002-3951-8961 | |
person.identifier.rid | T-6210-2018 | |
person.identifier.scopus-author-id | 23100224900 | |
rcaap.rights | openAccess | pt_PT |
rcaap.type | article | pt_PT |
relation.isAuthorOfPublication | 9653846d-d3c7-4760-b306-0723c927ab22 | |
relation.isAuthorOfPublication | 89899db7-a439-43c0-94bc-c738cee5324b | |
relation.isAuthorOfPublication | 1e019293-8ead-400e-a584-9d14eaa2bc4c | |
relation.isAuthorOfPublication.latestForDiscovery | 9653846d-d3c7-4760-b306-0723c927ab22 |
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