New clinical trial offers hope for patients with rare immune disease IgG4-RD

A team led by Mass General Brigham researchers demonstrated that inebilizumab reduced the risk of symptoms by 87% in patients with the rare affliction known as immunoglobulin G4–related disease (IgG4-RD)

An international, phase 3 clinical trial led by investigators at Mass General Brigham could improve the treatment of a rare disease that can cause debilitating symptoms. The study, published in the New England Journal of Medicine, found that treatment with inebilizumab greatly reduced the symptoms of immunoglobulin G4–related disease (IgG4-RD), compared to placebo.

“This is a huge day in the history of this disease,” said lead author John Stone, MD, MPH, a rheumatologist in the Division of Rheumatology, Allergy, and Immunology at Massachusetts General Hospital, a founding member of the Mass General Brigham healthcare system. “We are thrilled to have worked so closely with patients to undertake a trial specifically focused on their disease, with the goal of finding a therapy that we hope will be approved shortly for them.”

IgG4-RD is estimated to affect fewer than 200,000 people in the United States. Although IgG4-RD is an orphan disease first recognized to be a unique condition in 2003, review of the medical literature as far back as the late 1800s confirms that the disease was around even then – and likely much longer. People who have the condition suffer from a build-up of immune cells that produce the IgG4 antibody in certain organs. The disease can affect any organ and frequently involves multiple organs, most commonly the pancreas, bile ducts, salivary glands,

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Categorized as Immunology

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