Landmark data, formidable excitement: IgG4-related disease comes of age
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I have just returned and am recovering from #ACR24, and although there is a universe of things happening in our field, I want to focus for a moment on a rare, marginalized disease with a patient population desperately seeking effective care.
Of course, I am talking about IgG4-related disease.
Although not a new disease, it is indeed a new clinical entity whose codification has arisen within the past 20 years. This reality begs the question of where all these patients have been in the preceding times, and why do rheumatologists have such limited experience them, to say nothing of the considerable number of providers with no direct experience at all.
Perhaps the answer lies — as is so common in our field — within the folk tale of the blind man and the elephant. IgG4-related disease is remarkably protean, with the capacity to insidiously affect a single organ — for example, presenting with an asymptomatic enlargement of a gland in the head or neck — or alternatively manifest as a widespread illness with multiorgan involvement mimicking cancer, disseminated infection or immune mediated inflammatory diseases such as vasculitis, sarcoidosis and beyond. The blind man is the first practitioner to see the swollen submandibular gland, to note recurrent abdominal pain of unknown etiology, to address new-onset back pain in a middle-aged man with a retroperitoneal mass, and on and on and on.
During this year’s ACR Convergence, I was privileged to participate in and chair a satellite symposium with John Stone, MD, MPH, a master clinician known for both elucidating and teaching us all about IgG4-related disease, based on his focus and experience with it dating back nearly 20 years. I was also privileged to engage with Arezou Khosroshahi, MD, who trained with Dr. Stone and now focuses on IgG4-related disease at Emory University. Finally, I was also privileged to finally meet and interact with Matthew Baker, MD, MS, clinical chief of rheumatology at Stanford University, and a rheumatologist who thrives in the rare disease space, with expertise in both IgG4 and sarcoidosis.
This symposium was packed yet highly interactive, and I could feel from our audience their collective concerns regarding diagnosis and long-term management, as well as the need to increase the collective confidence level among rheumatology practitioners in both these aspects.
Two other events — just before and during ACR Convergence — have also contributed formidably to the excitement surrounding IgG4-related disease, which I would like to bring to your attention.
First, on the evening before ACR Convergence even started, a landmark paper on a new therapeutic agent, inebilizumab (Uplizna, Amgen), a humanized IgG1 kappa monoclonal antibody specifically targeting CD19, was published in The New England Journal of Medicine. This paper definitively demonstrated that this agent both reduces the risk for flares, but also increases the likelihood of flare free remission at 1 year.
This is good news for patients for IgG4-related disease, but it is also axiomatic that they have to be diagnosed first to access such a targeted therapy.
Secondly, while spending time at the Cleveland Clinic booth in the ACR Convergence exhibit hall, meeting with visitors and old friends, I noted near opposite was a nonprofit organization I knew little about named IgG4ward. This group — as the first and only organization solely dedicated to IgG4-related disease advocacy, research, and support — strives to improve the lives of people living with this disease. They are spreading the word to all that the disorder is under-recognized but also, now more than ever, appears much closer to becoming more manageable once a diagnosis is made. As my dear friend and giant of the world of vasculitis, Gary Hoffman, MD, MS, MACR, used to say: “You can’t diagnose what you don’t suspect.”
That’s my take on igG4-related disease. What’s yours? Please share your thoughts with me at calabrl@ccf.org or at rheumatology@healio.com.
- Reference:
- Stone JH, et al. N Engl J Med. 2024;doi:10.1056/NEJMoa2409712.
- For more information:
- Leonard H. Calabrese, DO, is the Chief Medical Editor, Healio Rheumatology, and Professor of Medicine, Cleveland Clinic Lerner College of Medicine of Case Western Reserve University, and RJ Fasenmyer Chair of Clinical Immunology at the Cleveland Clinic.
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