American College of Rheumatology and the European League Against Rheumatism classification criteria for IgG4-related disease: an update for radiologists.

IgG4-related disease (IgG4-RD) is an immune-mediated, multiorgan, chronic inflammatory disease. The three-step classification criteria proposed in 2019 by the American College of Rheumatology and the European League Against Rheumatism (ACR/EULAR) encompass a wide range of clinical, radiological, serological, and histopathological findings. The ACR/EULAR proposed a three-step classification process, i.e., entry step, exclusion step, and scoring system. Radiologists need to know that the radiological findings observed in the five domains of the lacrimal and salivary glands, chest, pancreas and biliary ducts, kidney, and retroperitoneum are independently weighted with different points in the scoring system. A total score < 20 points indicates that the patient should not be classified as having IgG4-RD; conversely, a total score ≥ 20 points indicates that the patient should be classified as having IgG4-RD. In this review, the 2019 ACR/EULAR classification criteria are discussed, focusing on the interpretation of each radiological item, with the aim of applying them to the diagnosis of IgG4-RD in clinical practice.