IgG4-related disease management still reliant on prednisone as new therapies emerge
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DESTIN, Fla. — IgG4-related disease may be regarded as this century’s “greatest mimicker,” but upcoming therapies and discoveries are reasons for excitement, according to a speaker at the 2023 Congress of Clinical Rheumatology-East.
“I am so pleased to say that I am more excited about this disease than ever,” John Stone, MD, MPH, of Massachusetts General Hospital, told attendees during the hybrid meeting. “I feel like we are really on the cusp of a new era of learning what this disease can teach us. This is a marvelous disease to study. It’s a slowly moving disease so we can treat patients with targeted therapy and observe what happens to the immune system. We have already learned a great deal from studying the disease in that fashion.”
Researchers in Japan have referred to the disease as being similar to a crow flying through a dark night, Stone explained. In recent years, the curtain has been lifted on the mysteries of the disease and more diagnostic and therapeutic strategies have become more widely adopted.
“It has been a pleasure to witness over the last 15 years, the emergence of this crow into full light,” Stone said. “We now know this is a multi-organ disease; it can literally effect any organ in the body.”
Additionally, Stone said, considering and accounting for IgG4-related disease may be able to help clinicians tie together organ-specific ailments that were previously not considered to be linked.
Prednisone remains the standard therapy for IgG4-related disease in the U.S. and abroad. He noted that, in China and Japan, patients may be started on 40 mg of prednisone per day, with the goal of tapering patients to 5mg to 10 mg per day and maintaining that therapy. In the U.S. and Europe, Stone said, insurance database examinations have shown that about 80% of patients receive prednisone as the first and only line of therapy. Patients may begin therapy at 40 mg per day, with a taper to 0 mg per day over 2 to 3 months, Stone said. In all instances, patients experience a large rate of disease flares.
“We try to taper patients off, and we still see the very high percentage of flare rates,” Stone said. “The early writers of this disease did us a disservice when they talked how great prednisone was.”
There are, however, “a number” of targets that offer hope for the future of disease management, Stone said. CD-19 cell depleting therapy is one of the targets that is currently being investigated and may offer hope for the future.
“There is a very exciting molecule that really has a unique method of action,” Stone said. “It binds to both CD-19, B cells and many B-cell subsets, as well as to FC gamma and R2B.” The molecule obexelimab would work by downregulating, but not depleting, B cells. In the case of a global pandemic, Stone said, the “ready reversibility” of this therapy holds appeal. In addition, BTK inhibitors are being investigated for the treatment of IgG4-related disease, Stone said.
“There are more discoveries, lots more discoveries, and much better treatments on the way soon,” Stone said.
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Stone J. IgG4-related disease. Presented at The Congress of Clinical Rheumatology East; May. 4-7, 2023; Destin, Florida (hybrid meeting).
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