Abstract

The concept of IgG4-related disease (IgG4-RD) has recently been individualized in the early 2000s, but most of the organ involvements are known since more than 100 years. IgG4-RD is a non-malignant fibroinflammatory disorder, characterized by peculiar immunological and pathological abnormalities, which can affect virtually all organs or tissues. Diagnostic criteria have been proposed and have evolved rapidly, with general or organ specific criteria. An international and multidisciplinary group assembled by the American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) has recently developed and validated a set of classification criteria called 2019 ACR/EULAR classification criteria for IgG4-related disease. The objective of this review is to discuss the evolution from organ specific and general diagnostic criteria toward the 2019 ACR/EULAR classification criteria, as well as respective benefits and limits of these criteria. The use of the 2019 ACR/EULAR classification criteria will help to better define homogeneous group of IgG4-RD patients in future clinical, epidemiological and basic science research studies on the disease.

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