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Author(s):
Anisha Dua, MD, MPH, defines IgG4 and explains why IgG4-related disease is often difficult to diagnose, in part because it regularly affects a variety of organ systems.
In an interview with HCPLive, Anisha Dua, MD, MPH, associate professor at Northwestern University, discussed her Rheumatology Winter Clinical Symposium (RWCS) presentation, “State of the Art: IgG4-Related Disease: what’s new?”
IgG4 is an increasingly recognized subset of IgG, an immunoglobulin, or protein in the blood, which is associated with a variety of clinical manifestations, including autoimmune pancreatitis, head and neck involvement, and enlargement of the salivary and lacrimal glands. Because of this, it is often difficult to recognize the syndrome. In her presentation, Dua focuses on ways in which to identify IgG4-related diseases, including recently published criteria to help make the diagnosis.
As the condition is so rare, it’s difficult to conduct large clinical trials. Through increasing awareness, however, it’s become easier to obtain medications for IgG4-related disease. Historically, clinicians have been able to prescribe standard disease-modifying antirheumatic drugs (DMARDs), such as methotrexate, and smaller trials evaluating the efficacy of certain drugs have been conducted.
“A lot of this is prednisone responsive; but we know that steroids have a lot of side effects,” Dua explained. “[We’re] trying to figure out other types of medications that can get the disease under better control and there's some pretty good evidence for rituximab being able to control the disease long-term.”
Understanding the different clinical presentations is imperative to diagnosing IgG4-related diseases, as there isn’t a singular diagnostic test to determine the presence of these conditions. Rheumatologists are tasked with sorting through the “pieces of the patient’s story,” including clinical exams, laboratory results, and imaging pathology, to land on a diagnosis.
“Because it can affect so many different organ systems, I think the main goal is going to be to try to increase awareness amongst rheumatologists, and also our colleagues in other fields, to recognize it and send it our way so we can get it under control,” Dua emphasized.
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