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The Half-Life of the Truth

Philip Seo, MD, MHS  |  Issue: July 2020  |  July 15, 2020

My fellow was laughing at me.

By itself, I don’t think this was an unusual occurrence. I am quite certain that my fellows laugh at me all the time. That said, when such laughter is called for, I am accustomed to a certain protocol being observed. In general, I expect the laughter to be contained, discreet. To be blunt, I expect my trainees to laugh at me behind my back. Having a trainee laugh at my front was a novel experience.

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I was working with said fellow on the inpatient consult service. We had been asked to evaluate an older patient with a long-standing diagnosis of scleroderma who had developed digital gangrene. If this were a question on a multiple choice test, the answer would be epoprostenol.

In patients with digital ischemia, prostacyclins may improve circulation at both the microvascular and macrovascular levels, by inducing vasodilation, increasing intra­cellular cyclic adenosine monophosphate (cAMP) and inhibiting smooth muscle proliferation. Clinically, these benefits can be profound; I remember life before epoprostenol, and I can tell you that it was worse. It seems to be particularly good for helping patients with the pain associated with digital ischemia, which is even more heartbreaking than the loss of tissue.

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This particular patient was admitted to our main hospital, where, for reasons that defy both good medicine and good logic, epoprostenol is unavailable to patients with scleroderma. Because the drug is formally indicated only for the treatment of pulmonary hypertension, in my hospital, its use has been restricted only to patients with that diagnosis. Ironically, some patients with ischemic digits are too healthy to merit the drugs they need.

In those cases, we tweak. We use calcium channel blockers and nitrates, increase the ambient temperature and work on pain control, in hopes that some combination of all of these nudges will push the patient in the right direction.

This particular patient also had a long-standing diagnosis of anemia from gastric antral vascular ectasia (GAVE), which is sometimes referred to as watermelon stomach. On endoscopy, it is described as “rough parallel folds and dilated blood vessels departing from the pylorus and converging in the gastric antrum”; in a moment of whimsy, you could see why this might make a hungry gastroenterologist think of a watermelon. Although GAVE can be found in a number of medical conditions, including cirrhosis, renal failure, chronic pulmonary disease and diabetes, a rheuma­tologist would most often associate it with a diagnosis of scleroderma. GAVE is more than a piece of rheumatic trivia, because the ectatic blood vessels tend to bleed, leading to a chronic iron-deficiency anemia.

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Filed under:Education & TrainingOpinionResearch RheumRheuminationsSpeak Out Rheum Tagged with:Research

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