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ACR AND EULAR PRESENT DRAFT OF THE FIRST CLASSIFICATION CRITERIA FOR IgG4-RELATED DISEASE
Newswise — CHICAGO – The American College of Rheumatology (ACR) and the European League Against Rheumatism (EULAR) will present a draft of the Classification Criteria for IgG4-Related Disease, the first classification criteria developed for this recently recognized disease, during a session at the 2018 ACR/ARHP Annual Meeting this week at McCormick Place in Chicago. The classification criteria are currently under review by the ACR and EULAR for endorsement.
The session will take place on Wednesday, Oct. 24, at 9:00 AM (CT) in W375b and will include a presentation by John H. Stone, MD, MPH, Director of Clinical Rheumatology and the Edward A. Fox Chair in Medicine at Massachusetts General Hospital, who directed an international panel of experts who developed the new criteria. Dr. Stone will also discuss the new criteria at a press conference that will take place on Tuesday, Oct. 23, at 12:30 PT (CT) in W175a.
IgG4-Related Disease (IgG4-RD) is an immune-mediated disease that may affect different organ systems and often mimics other diseases, Sjögren’s syndrome, pancreatic cancer, granulomatosis with polyangiitis (GPA), giant cell arteritis and systemic lupus erythematosus (SLE or lupus), says Dr. Stone. Only recognized about a decade ago, 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.
“Many of these patients have undergone radical surgical procedures, such as modified pancreatectomies, because they were believed to have cancer. Their physicians did not recognize that what they had in fact was an inflammatory disorder that is a highly treatable condition,” he says.
“Many times IgG4-RD has been missed for months or even years, and a great deal of damage can occur in these patients. Classification criteria for IgG4-RD are badly needed at this time, as researchers around the world are conducting clinical trials for therapies to treat this disease.”
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.
In the criteria, classifying patients with IgG4-RD is a three-step process that carefully assesses data from four domains. There is no single diagnostic test for this disease at this time, says Dr. Stone.
“In order to arrive at the diagnosis of IgG4-RD, the clinical imperative is to synthesize information from the patient’s clinical presentation, the results of blood tests or serology, the radiological findings and the pathology data. All of these must be integrated and make sense in the context of IgG4-RD,” says Dr. Stone. “Few other diseases require such careful synthesis of different types of information in order to arrive at the correct diagnosis.”
The Classification Criteria for IgG4-Related Disease were validated in a large cohort of patients and demonstrated excellent test performances. Dr. Stone feels they should be a highly useful contribution to future investigations in this disease, and ultimately help improve the lives of patients with IgG4-RD.
“IgG4-RD has started to come out of the woodwork. Rheumatologists are seeing it more often in their clinical practice and being referred patients who have it,” he says. “The development of this first classification criteria is incredibly timely, and will also help rheumatologists recognize patients in their own practice who have the disease. Although it remains a challenging disease to diagnose, we have come a very long way in a short time in understanding the pathophysiology of this disease.”
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About the ACR/ARHP Annual Meeting
The ACR/ARHP Annual Meeting is the premier meeting in rheumatology. With more than 450 sessions and thousands of abstracts, if offers a superior combination of basic science, clinical science, tech-med courses, career enhancement education and interactive discussions on improving patient care. For more information about the meeting, visit https://www.rheumatology.org/Annual-Meeting, or joint the conversation on Twitter by following the official #ACR18 hashtag.
About the American College of Rheumatology
The American College of Rheumatology is an international medical society representing over 9,400 rheumatologists and rheumatology health professionals with a mission to empower rheumatology professionals to excel in their specialty. In doing so, the ACR offers education, research, advocacy and practice management support to help its members continue their innovative work and provide quality patient care. Rheumatologists are experts in the diagnosis, management and treatment of more than 100 different types of arthritis and rheumatic diseases. For more information, visit www.rheumatology.org.
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