Klin Onkol 2021; 34(2): 92-102. DOI: 10.48095/ccko202192.
Background: IgG4-related disease (IgG4-RD) is a non-malignant, chronic, immune-related disease. It was first recognized as a distinct disease in 2012 and the first classification criteria were published in 2020. This new entity can cause fibroinflammatory lesions in nearly any organ. It often presents as a multi-organ disease and can be confused with malignancy, infection or other immune-mediated conditions. Although the disease could affect virtually any organ, there are strong predilections for certain organs: the major salivary glands, the orbits and lacrimal glands, the pancreas and biliary tree, the lungs, the kidneys, the aorta and retroperitoneum, the meninges and the thyroid gland. Purpose: Correlation among clinical, serologic, radiologic and pathologic data is required for establishing IgG4-RD. We sum up the newest information necessary for the diagnosis.
