Immunoglobin G4-Related Diseases

IgG4-related disease (IgG4-RD) is characterised by its pathological, serological, and clinical features, and its recognition is gradually increasing in the literature. This disease is generally observed in middle-aged males and older groups. IgG4 positive plasma cells and T lymphocytes infiltrate various organs, characterised by the etiology of an unknown disease. Almost all organs of the body are affected, but those most frequently affected are the pancreas, bile duct, gallbladder, salivary glands, retroperitoneal region, kidneys, lungs, and the prostate. The histopathological features of this disease are storiform fibrosis and obliterative phlebitis. Corticosteroids are useful in the treatment of this disease. This study aims to increase the awareness of IgG4-related disease through a review of the recent literature.