Fibrose retroperitoneal como apresentação clínica da doença relacionada ao IGG4: revisão bibliográfica

Abstract

Immunoglobulin G4-related disease (DR-IgG4) is an uncommon fibroinflammatory condition that can affect multiple organs and shares pathological, serological and clinical characteristics with several other diseases, which makes its diagnosis a major clinical challenge. Among the clinical presentations described, retroperitoneal fibrosis (RPF) stands out, characterized by a chronic inflammatory reaction in the retroperitoneum with consequent formation of fibrotic tissue. The characteristic histopathological findings are dense lymphoplasmacytic infiltrations rich in IgG4-positive plasma cells, obliterative phlebitis, storiform fibrosis and, often, elevated serum IgG4 concentrations. Twelve scientific articles available in the PubMed, Scopus and Google Scholar databases were selected from 2019 to 2024, highlighting the main findings of each study, seeking to identify and compare their convergences and divergences. Steroid therapy is the main treatment option for patients with IgG4-related Retroperitoneal Fibrosis. Immunosuppressive drugs (Cyclophosphamide, Mycophenolate, Methotrexate) can be used to spare corticosteroids. Although its first description was in 2003, the literature shows the important role of IgG4-related disease as a differential diagnosis of infiltrative lesions of the retroperitoneum. Its insidious clinical picture and ability to mimic other conditions makes diagnosis challenging, requiring correlations between clinical, radiological and histopathological data.