A Rare Case of Retroperitoneal Fibrosis Caused By IgG4-Related Disease

Immunoglobulin G4-related disease (IgG4-RD) is an inflammatory condition causing tissue fibrosis, resulting in underlying organ destruction. IgG4-RD can affect multiple tissues throughout the body, causing a wide variety of conditions. Our case confirms the autoimmune component of the disease by its brisk response to immunosuppressive therapy and highlights the importance of early recognition and diagnosis in order to start treatment before the development of irreversible end-organ injury.