[IGG4-related disease. Report of 52 patients].

Autor:	Cuéllar MC; Sección de Reumatologia, Hospital del Salvador, Universidad de Chile, Santiago, Chile., Gutiérrez M; Hospital Naval Almirante Nef. Escuela de Medicina, Universidad de Valparaíso, Viña del Mar, Chile., Herrera A; Pontificia Universidad Católica de Chile, Santiago, Chile., Elgueta F; Hospital Clínico Universidad de Chile, Santiago, Chile., Wurmann P; Hospital Clínico Universidad de Chile, Santiago, Chile., Badilla N; Sección de Reumatologia, Hospital del Salvador, Universidad de Chile, Santiago, Chile., Mansilla B; Pontificia Universidad Católica de Chile, Santiago, Chile., Basualdo J; Sección de Reumatologia, Hospital del Salvador, Universidad de Chile, Santiago, Chile., Vega J; Hospital Naval Almirante Nef. Escuela de Medicina, Universidad de Valparaíso, Viña del Mar, Chile., Erlij D; Sección de Reumatologia, Hospital del Salvador, Universidad de Chile, Santiago, Chile., Labarca C; Facultad de Medicina Clínica Alemana, Clínica Alemana de Santiago, Universidad del Desarrollo, Santiago, Chile., Vergara C; Hospital de Quillota, Valparaíso, Chile., Mezzano V; Pontificia Universidad Católica de Chile, Santiago, Chile., Méndez I; Departamento de Salud Ocupacional, Corporación Nacional del Cobre, Santiago, Chile., Stange L; Pontificia Universidad Católica de Chile, Santiago, Chile., Michalland S; Sección de Reumatologia, Hospital del Salvador, Universidad de Chile, Santiago, Chile., Silva F; Facultad de Medicina Clínica Alemana, Clínica Alemana de Santiago, Universidad del Desarrollo, Santiago, Chile., Jara A; Pontificia Universidad Católica de Chile, Santiago, Chile., Goecke A; Hospital Clínico Universidad de Chile, Santiago, Chile.
Jazyk:	Spanish; Castilian
Zdroj:	Revista medica de Chile [Rev Med Chil] 2022 Jun; Vol. 150 (6), pp. 705-710.
DOI:	10.4067/S0034-98872022000600705
Abstrakt:	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.
Databáze:	MEDLINE
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