| Revue: | Revista médica de Chile |
| Base de datos: | PERIÓDICA |
| Número de sistema: | 000455019 |
| ISSN: | 0034-9887 |
| Autores: | Cuéllar, María C1 Gutiérrez, Miguel2 Herrera, Alejandra3 Elgueta, Fabián4 Wurmann, Pamela4 Badilla, Natalia1 Mansilla, Bellanides3 Basualdo, Javier1 Vega, Jorge2 Erlij, Daniel1 Labarca, Cristian5 Vergara, Cristian8 Mezzano, Verónica3 Méndez, Ignacio6 Stange, Lilith3 Michalland, Susana1 Silva, Francisco5 Jara, Aquiles3 Goecke, Annelise4 Burgos, Paula3 Iruretagoyena, Mirentxu3 Fernández, Cristina7 Landeros, Carolina1 Neira, Óscar1 |
| Instituciones: | 1Hospital del Salvador, Sección de Reumatologia, Santiago de Chile. Chile 2Hospital Naval Almirante Nef, Viña del Mar. Chile 3Pontificia Universidad Católica de Chile, Santiago de Chile. Chile 4Universidad de Chile, Hospital Clínico , Santiago de Chile. Chile 5Universidad del Desarrollo, Clínica Alemana de Santiago, Santiago de Chile. Chile 6Corporación Nacional del Cobre, Departamento de Salud Ocupacional, Santiago de Chile. Chile 7Instituto Nacional del Tórax, Santiago de Chile. Chile 8Hospital de Quillota, Valparaíso. Chile |
| Año: | 2022 |
| Periodo: | Jun |
| Volumen: | 150 |
| Número: | 6 |
| Paginación: | 705-710 |
| País: | Chile |
| Idioma: | Español |
| Tipo de documento: | Artículo |
| Enfoque: | Analítico, descriptivo |
| Resumen en inglés | 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 |
| Disciplinas: | Medicina |
| Palabras clave: | Inmunología, Trastorno fibro-inflamatorio, Enfermedad de la IgG4, Manifestaciones clínicas, Corticosteroides, Inmunosupresión |
| Keyword: | Immunology, Fibro-inflammatory disorder, IgG4 disease, Clinical manifestations, Corticosteroids, Immunosuppression |
| Texte intégral: | Texto completo (Ver HTML) Texto completo (Ver PDF) |
