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Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos

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dc.contributor.authorCuéllar, María C.
dc.contributor.authorGutiérrez, Miguel
dc.contributor.authorHerrera, Alejandra
dc.contributor.authorElgueta, Fabián
dc.contributor.authorWurmann, Pamela
dc.contributor.authorBadilla, Natalia
dc.contributor.authorMansilla, Bellanides
dc.contributor.authorBasualdo, Javier
dc.contributor.authorVega, Jorge
dc.contributor.authorErlij, Daniel
dc.contributor.authorLabarca, Cristian
dc.contributor.authorVergara, Cristian
dc.contributor.authorMezzano, Verónica
dc.contributor.authorMéndez, Ignacio
dc.contributor.authorStange, Lilith
dc.contributor.authorMichalland, Susana
dc.contributor.authorSilva, Francisco
dc.contributor.authorJara, Aquiles
dc.contributor.authorGoecke, Annelise
dc.contributor.authorBurgos, Paula
dc.contributor.authorIruretagoyena, Mirentxu
dc.contributor.authorFernández, Cristina
dc.contributor.authorLanderos, Carolina
dc.contributor.authorNeira Quiroga, Oscar
dc.date.accessioned2023-12-13T16:26:17Z
dc.date.available2023-12-13T16:26:17Z
dc.date.issued2022
dc.description.abstractBackground: IgG4-related disease (IgG4 RD) is an immune-mediated fibro-inflammatory disorder, with tissue infiltration of IgG4+ plasma cells. It causes pseudotumors, tumors, and a wide spectrum of clinical manifestations. Aim: To report the clinical, laboratory, histopathological and treatment characteristics of a group of Chilean patients with IgG4 RD. Material and Methods: Review of medical records of 52 patients aged 18 to 76 years with IgG4 RD seen at six medical centers. Results: Elevated IgG4 serum levels (> 135 mg/dl) were found in 18 of 44 (41%) patients. There was histological confirmation of the disease in 46 patients. The most common sites of involvement were lungs, eyes and kidneys. Eighteen (35%) patients had only one organ involved, 34 (65%) patients had two organs and 13 (25%) patients had three or more organs. The involvement of two organs was significantly more common in men (p < 0.05). In patients with only one organ involvement, the most frequent location was orbital and meningeal. All patients with kidney or lung disease had multiorgan involvement. All patients received corticosteroid therapy, 67% synthetic immunosuppressants, and 16% rituximab. Conclusions: ER-IgG4 can affect any tissue. Multiorgan involvement was more common in this series, with preference for lungs, eyes and kidneys. An excellent response to steroids is characteristic of the disease, but with a high relapse rate that requires additional immunosuppression.
dc.description.versionVersión aceptada
dc.identifier.citationCuéllar María C., Gutiérrez Miguel, Herrera Alejandra, Elgueta Fabián, Wurmann Pamela, Badilla Natalia et al . Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos. Rev. méd. Chile [Internet]. 2022 Jun [citado 2023 Dic 13] ; 150( 6 ): 705-710. Disponible en: http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872022000600705&lng=es. http://dx.doi.org/10.4067/S0034-98872022000600705.
dc.identifier.doihttps://doi.org/10.4067/S0034-98872022000600705
dc.identifier.urihttps://repositorio.udd.cl/handle/11447/8164
dc.language.isoes
dc.subjectImmunoglobulin G4-Related Disease
dc.titleEnfermedad relacionada a IgG4. Serie clínica de pacientes chilenos
dc.title.alternativeIGG4-related disease. Report of 52 patients
dc.typeArticle
dcterms.sourceRevista Médica de Chile
dspace.entity.typePublication

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