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August 18, 2017
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IgG4 Disease and Zen: Is There Another Association?

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Leonard H. Calabrese

I hope, dear reader, that you are not tiring of my return to Eastern thought to introduce yet another major disease story in Healio Rheumatology, but I will defend myself by saying that timeless wisdom is hard to beat. Having apologized, I will explain this association in a moment, but let me first tell you how pleased I am to host this stimulating discussion on immunoglobin G4 or IgG4 disease with John H. Stone, MD; Antoine G. Sreih, MD; and Corey Perugino, DO.

As practitioners, it is not often that we are privileged to witness the evolution of a new disease construct within the timeframe of few years. Think about it. Aside from notable environmental diseases caused by exposures, such as nephrogenic systemic fibrosis, a byproduct of the era of MRI which I have had the wonder to observe during my career, it is uncommon to unravel prior disease constructs into a new way of thinking so rapidly.

being mindful in medicine

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We Now See Cohesion

From my parochial perspective, I suppose I would add the evolution of antineutropil cytoplastic antibody (ANCA)-associated vasculitis as a construct to this list. Previously, we thought about this group of vasculitic disorders as somewhat disparate; however, we now see cohesion. The ANCA story, however, seems more to me like a slow-moving freight train, while the IgG4 story is a Japanese bullet train. In addition, IgG4 disease is seemingly everywhere, albeit it is still a rare disease.

I have had a long interest in retroperitoneal fibrosis and have cared for hundreds of patients. Yet, I now feel like I must have had blinders on when I cared for patients with retroperitoneal fibrosis and orbital pseudotumor, Riedell’s thyroiditis, atypical lymphandenopathic disease, mediastinal fibrosis and spinal cord invasion among other complications. Why didn’t I see these disorders differently, as linked entities or at the minimum ask myself, “What’s up with this?” Now RPF is centrally ensconced in the IgG4 spectrum and it seems so clarion that there must have been a cohesion among these disparate complications that I and others repeatedly observed, yet failed to see, 20 years or even 10 years ago.

Mindfulness in Medicine

In Zen, it is said that to be wise one must have a beginner’s mind, which means that when unjaded you will be more likely to ask the obvious questions as a child does when confronted with a new encounter. In medicine, we muse at the old joke of the medical student who nails the early diagnosis of pancreatic cancer in a patient with new onset non-descript abdominal pain. His explanation of how he accomplished this Oslerian feat, of course, was “What else causes abdominal pain?”

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The Zen monk Shunryu Suzuki puts it more simply but elegantly in his book Zen Mind, Beginner’s Mind. “In the beginner’s mind there are many possibilities, in the expert’s mind there are few,” he writes. I believe this is a concept we should hold close through a life in medicine so we do not wind up walking through our careers on automatic pilot. Being mindful in medicine is no new wave contrivance. It is all about seeing wisely, not just seeing a lot.

What other diseases have you re-thought during your career? Please share your experiences by emailing calabrl@ccf.org. You can also follow me on Twitter @LCalabreseDO or @HealioRheum. Thanks for reading.

Disclosure: Calabrese reports he is a consultant for Genentech, Pfizer, Bristol-Myers Squibb, Glaxo SmithKline, Sanofi, Jansen and AbbVie; and is on the speakers bureau for Genentech, AbbVie and Bristol-Myers Squibb and Crescendo Bioscience.