Empowered Patient Podcast New Biologic Drug for Rare IgG4-Related Disease with Dr. Adam Kilian St. Louis University School of Medicine
Dr. Adam Kilian, Director of the Rheumatology Fellowship Training Program at the St. Louis University School of Medicine, focuses on IgG4-related disease, a rare multi-organ disease that has only recently been recognized due to significant diagnostic challenges. The MITIGATE trial is a landmark study that demonstrated that the first FDA-approved treatment, UPLIZNA from Amgen, provides an effective, targeted, steroid-free therapy. The approval of this drug is driving awareness in the medical community about IgG4-RD and whether the disease should be considered after inconclusive results for other suspected conditions.
Adam explains, "IgG4-related disease is a rare disease that affects many organ systems. It's a chronic systemic, fibroinflammatory disease that can affect almost any organ system. And it will usually present with these tumor-like inflammatory masses that can cause scarring and lead to organ failure."
"Our understanding of it continues to evolve, and it's had a really interesting story over the last century because IgG4-related disease affects so many different organ systems. Over the past century, there were actually many different diagnoses that were recognized, which now all fall under the umbrella of IgG4-related disease. Different scientists and physicians around the world over the years recognized the different manifestations of the disease in the pancreas, in the lymph nodes, in the head and neck, in the kidneys, or in the blood vessels. And it's only been in the last 20 years or so that we have recognized that actually all of these different disease entities from the last century are actually all manifestations of this disease, IgG4, IgG4-related disease."
"The clinical trial is called the MITIGATE trial, and it's a very seminal clinical trial in rheumatology from the last several years. This was the first-ever phase three double-blind, placebo-controlled, randomized controlled trial in IgG4-related disease. This was a huge trial. It was global. It was conducted in 22 countries with multiple specialties coordinating this trial. It was 52 weeks long, and its purpose was to evaluate the effectiveness and the safety of UPLIZNA in patients who have IgG4-related disease."
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