• María C Cuéllar, Miguel Gutiérrez, Alejandra Herrera, Fabián Elgueta, Pamela Wurmann, Natalia Badilla, Bellanides Mansilla, Javier Basualdo, Jorge Vega, Daniel Erlij, Cristian Labarca, Cristian Vergara, Verónica Mezzano, Ignacio Méndez, Lilith Stange, Susana Michalland, Francisco Silva, Aquiles Jara, and Annelise Goecke.
• Sección de Reumatologia, Hospital del Salvador, Universidad de Chile, Santiago, Chile.
• Rev Med Chil. 2022 Jun 1; 150 (6): 705710705-710.

BackgroundIgG4-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.AimTo report the clinical, laboratory, histopathological and treatment characteristics of a group of Chilean patients with IgG4 RD.Material And MethodsReview of medical records of 52 patients aged 18 to 76 years with IgG4 RD seen at six medical centers.ResultsElevated 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.ConclusionsER-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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