PMR advances highlight need for earlier, collaborative diagnosis in PC, rheumatology
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As I have said many times, rheumatology is the most interprofessional and collaborative specialty, for by its very nature our diseases cross every barrier in their clinical manifestations of target organ involvement, and lines of care.
Recently, a lot of attention has been directed to one of our specialty’s most unique and gratifying-to-care-for conditions — polymyalgia rheumatica, or PMR. Although we so often toil to care for chronic and poorly understood disorders that require a lifetime of therapy, often with incomplete responses and requiring cycling of numerous therapeutics, PMR stands alone. The disease is distinctive in so many ways, but to me what is most striking is its capacity to affect so profoundly the quality of life of an individual patient, often in what seems like a blink of an eye.
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On the other hand, it masquerades as so many diseases from the mundane to the rare.
I can recall a patient who was previously labeled as having atypical stiff person syndrome, and referred to me for his unexplained ESR of 100 mm/hr. It was so severe that he had to build a ramp to get into his house because he could no longer climb the three stairs to his doorway. After sorting things out and committing to a therapeutic trial of prednisone, by the next morning he was dancing his way down the stairs. Regardless of the inherent uncertainties, when PMR is accurately diagnosed and treated the results are gratifying to both patient and practitioner.
Now of course there are dark sides to the overall management of PMR, much of which is elegantly discussed by Sebastian Sattui, MD, MS, in our focused interview this month, “‘Communication and collaboration’ essential to managing polymyalgia rheumatica, GCA.”
Although most practitioners abide with his major tenet that early diagnosis and referral are key issues in PMR, Dr. Sattui supports this with a growing body of literature bolstering these concerns.
Primary care physicians are often put in awkward situations when confronting new onset possible PMR.
The patient is suffering and the potential treatment (ie, glucocorticoids) is potentially capable of relieving this suffering almost immediately, but the path is often complex with diagnostic challenges looming at every turn. Is the diagnosis acute? Could this be one of several “must rule out” conditions like cancer or infection? What is the best dose of glucocorticoids to start? Finally, what should be done right now while I fight to get this patient in to see a rheumatologist who has a long, long wait list?
Data from multiple studies support that the majority of patients with PMR are never seen by a rheumatologist and the rate of diagnostic error is too high. We clearly need more fast-track clinics or even informal systems to address such patient needs. What about an AI algorithm for starters?
Finally, the elephant in the room for the past 70 years has been the long term toxicity of glucocorticoids, which is particularly concerning in this vulnerable patient population. Importantly, on March 1 of this year the FDA approved sarilumab (Kevzara; Sanofi, Regeneron), the first biologic for the treatment of adult patients with PMR who have an inadequate response to corticosteroids (CS) or are unable to tolerate CS taper.
I consider this approval potentially as impactful as the approval of tocilizumab (Actemra, Genentech) for giant cell arteritis in 2017, as our experience and supporting evidence for the effectiveness of current steroid-sparing agents for PMR is particularly weak. Over the past few years our profession has turned its focus across all immune mediated diseases to reduce or eliminate the use of glucocorticoids as chronic therapies. Although many questions remain unanswered, including how to optimize patient selection and duration, these are welcome uncertainties given the potential to improve our care and caring of PMR.
That’s my take. What’s yours? Please share your thoughts with me at calabrl@ccf.org or at rheumatology@healio.com.
- 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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