Enfermedad relacionada con IgG4

Abstract

IgG4-related disease (IgG4-RD) is an immune-mediated disorder of a fibroinflammatory nature that can affect practically any organ. Some of the most common manifestations are glandular involvement, orbital pseudotumor, pancreatic involvement, and retroperitoneal fibrosis. The 2019 ACR/EULAR classification criteria for IgG4-RD, based on clinical and histologic findings, may be useful for diagnosis. Corticosteroids are the mainstay of treatment, although different immunosuppressive drugs are under investigation with mixed results.