Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos
Background: 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.
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Sociedad Médica de Santiago
2022
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oai:scielo:S0034-988720220006007052022-12-18Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenosCuéllar,María C.Gutiérrez,MiguelHerrera,AlejandraElgueta,FabiánWurmann,PamelaBadilla,NataliaMansilla,BellanidesBasualdo,JavierVega,JorgeErlij,DanielLabarca,CristianVergara,CristianMezzano,VerónicaMéndez,IgnacioStange,LilithMichalland,SusanaSilva,FranciscoJara,AquilesGoecke,AnneliseBurgos,PaulaIruretagoyena,MirentxuFernández,CristinaLanderos,CarolinaNeira,Óscar Immunoglobulin G4-Related Disease Background: 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.info:eu-repo/semantics/openAccessSociedad Médica de SantiagoRevista médica de Chile v.150 n.6 20222022-06-01text/htmlhttp://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872022000600705es10.4067/S0034-98872022000600705 |
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Cuéllar,María C. Gutiérrez,Miguel Herrera,Alejandra Elgueta,Fabián Wurmann,Pamela Badilla,Natalia Mansilla,Bellanides Basualdo,Javier Vega,Jorge Erlij,Daniel Labarca,Cristian Vergara,Cristian Mezzano,Verónica Méndez,Ignacio Stange,Lilith Michalland,Susana Silva,Francisco Jara,Aquiles Goecke,Annelise Burgos,Paula Iruretagoyena,Mirentxu Fernández,Cristina Landeros,Carolina Neira,Óscar |
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Cuéllar,María C. Gutiérrez,Miguel Herrera,Alejandra Elgueta,Fabián Wurmann,Pamela Badilla,Natalia Mansilla,Bellanides Basualdo,Javier Vega,Jorge Erlij,Daniel Labarca,Cristian Vergara,Cristian Mezzano,Verónica Méndez,Ignacio Stange,Lilith Michalland,Susana Silva,Francisco Jara,Aquiles Goecke,Annelise Burgos,Paula Iruretagoyena,Mirentxu Fernández,Cristina Landeros,Carolina Neira,Óscar Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos |
author_facet |
Cuéllar,María C. Gutiérrez,Miguel Herrera,Alejandra Elgueta,Fabián Wurmann,Pamela Badilla,Natalia Mansilla,Bellanides Basualdo,Javier Vega,Jorge Erlij,Daniel Labarca,Cristian Vergara,Cristian Mezzano,Verónica Méndez,Ignacio Stange,Lilith Michalland,Susana Silva,Francisco Jara,Aquiles Goecke,Annelise Burgos,Paula Iruretagoyena,Mirentxu Fernández,Cristina Landeros,Carolina Neira,Óscar |
author_sort |
Cuéllar,María C. |
title |
Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos |
title_short |
Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos |
title_full |
Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos |
title_fullStr |
Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos |
title_full_unstemmed |
Enfermedad relacionada a IgG4. Serie clínica de pacientes chilenos |
title_sort |
enfermedad relacionada a igg4. serie clínica de pacientes chilenos |
description |
Background: 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. |
publisher |
Sociedad Médica de Santiago |
publishDate |
2022 |
url |
http://www.scielo.cl/scielo.php?script=sci_arttext&pid=S0034-98872022000600705 |
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