CPD 90: POLYMYALGIA RHEUMATICA Continuing Professional Development
CPD 60 Second Summary Polymyalgia rheumatica (PMR) is a common inflammatory rheumatic disease which demonstrates a strong association with aging. In Ireland, 2% of the population will experience the condition at some time during their life. Characteristic symptoms constitute bilateral hip and shoulder stiffness with an early morning predominance. Inflammatory markers (ESR and CRP) are generally elevated, but not always markedly so. Many other conditions can present with polymyalgia-like symptoms, with potential negative consequences if missed, therefore a thorough clinical assessment at initial presentation is crucial. Symptom response to relatively modest doses of glucocorticoids, for example 15mg prednisolone, is usually dramatic. Long-term glucocorticoids are needed however, with a minimum of one year duration. Difficulties therefore arise due to the challenge of maintaining a good clinical response while minimising glucocorticoid related adverse events. Careful navigation of these challenges has the potential to improve long term outcomes for people with PMR and close clinical monitoring is essential. There are several treatment options which now have evidence of efficacy for relapsing or refractory disease, including methotrexate, tocilizumab, and rituximab. Adjunctive treatment for bone and gastro protection is crucial, as are patient education and careful management of emergent treatment or disease related complications.
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AUTHOR: Dr Richard Conway, Consultant Rheumatologist, St. James Hospital; Clinical Associate Professor, Trinity College Dublin Richard Conway graduated from the Royal College of Surgeons in Ireland in 2006. He completed rheumatology and general internal medicine training in Ireland in 2014, and a PhD in giant cell arteritis at University College Dublin in 2017. He is currently a consultant rheumatologist and physician at St. James’s Hospital and a clinical associate professor at Trinity College Dublin. He is the author of more than 150 peer-reviewed publications and 3 book chapters. His research interests include interstitial lung disease in systemic rheumatic diseases, vasculitis, and polymyalgia rheumatica.
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4 previous steps, log and record your findings. Published by HPN. Copies can be downloaded from www.irishpharmacytraining.ie Disclaimer: All material published is copyright, no part of this can be used in any other publication without permission of the publishers and author. Galapagos UK, has no editorial oversight of the CPD programmes included in these modules.
Management and Treatment of Polymyalgia Rheumatica Introduction Polymyalgia Rheumatica (PMR) is an inflammatory rheumatic condition. It has a striking age predilection, virtually never occurring prior to age 50 and becoming more common until a peak in the 70’s with a plateau thereafter. PMR is a common disease, with an incidence equivalent to rheumatoid arthritis (RA). It demonstrates a marked geographic preponderance being strikingly more common in Northern European populations than in other parts of the world. This association seems to be genetically based rather than due to physical location with emigrant populations maintaining the risk of their ancestral origin. The lifetime risk of PMR in Ireland (extrapolated from similar populations) is 2.5% for women and 1.5% for men.1 The cause of PMR is unknown; the marked age association suggests a key role for immunosenescence. It is hypothesised that a particular individual’s immune system may be primed, by aging and/or other factors, to develop PMR, and then a subsequent second hit triggers the disease. The nature of this trigger has been elusive, and this may reflect the fact that there is no specific trigger; rather it may be the case that anything that
stimulates the immune system can act in this role, this would correlate with the wide variety of infectious agents mooted as triggers, and indeed with the more controversial association with vaccines. Clinical Presentation PMR is very much a disease defined by the word “usually”. While there is a classic presentation, the individual components of which are seen in the majority of patients, the absence of these features does not rule out the diagnosis. By virtue of how common PMR is, it becomes relatively frequent to see patients with atypical presentations, where we run the risk of erroneously ruling out PMR. PMR typically presents with a sudden onset of symptoms. This can be dramatic; a patient can go to bed perfectly well and wake the subsequent morning unable to move. Patients will often report that “I became an old woman/ man overnight”. The symptoms are most often described as an ache or stiffness but may be referred to as pain. The typical sites affected are the proximal limb muscles – the shoulders and hips, and the neck. However, it is a myth that distal involvement rules out PMR, up to 50% of patients will have
involvement of the hands, wrists, or knees; frequently this is mild but can occasionally predominate. PMR is classically a bilateral and symmetric condition. At initial onset one side may be affected in isolation, however it rapidly progresses to involve both sides. While most patients will present with both shoulders and hips affected, they may not be equally involved, and a substantial minority of patients will report only shoulder or hip involvement in isolation. Early morning predominance of symptoms is characteristic, this is described as early morning stiffness – a shared feature of all inflammatory arthritis conditions. Patients will often also become stiff to a lesser degree after sitting for prolonged periods – not infrequently on the car journey to their clinic appointment or sitting in the waiting room! Patients with more severe symptoms will often not manifest the characteristic improvement during the day, they will wake with early morning stiffness which will then continue until they go to bed again. Systemic symptoms such as weight loss, malaise, and anorexia are common. Fever however is unusual in isolated PMR and should prompt a search for one of the PMR mimics.
