The American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) released the 2019 ACR/EULAR Classification Criteria for IgG4-Related Disease. It is the first criteria developed specifically for this recently recognized disease.
A draft of the criteria was presented during the 2018 ACR/ARP Annual Meeting in Chicago. Since that time, the criteria team performed a second validation study, which confirmed the high sensitivity and specificity that was found in the first validation study.
IgG4-Related Disease (IgG4-RD) is an immune-mediated disease that may affect different organ systems and often mimics other diseases like Sjögren's syndrome, pancreatic cancer, granulomatosis with polyangiitis (GPA), giant cell arteritis (GCA) and systemic lupus erythematosus (SLE or lupus). Only recognized in the last 10 to 15 years, IgG4-RD can cause fibro-inflammatory lesions in nearly any organ or multiple organs. Estimates suggest that IgG4-RD affects 180,000 people in the United States and many more worldwide.
Classification criteria allow researchers to accurately identify patients for inclusion in clinical, epidemiologic and basic investigations. The panel of experts who developed the new classification criteria included investigators from rheumatology and other specialties from five continents, reflecting the worldwide impact of this disease.