When PMR Strikes Young, It Hits Hard Save
Narratives around polymyalgia rheumatica (PMR) often centre around “older people” or even “elderly”. And, indeed, the peak of the age distribution is in the mid-70s – not that everyone that age considers themselves old. But often what comes with that narrative is a focus on palliating the symptoms in the here-and-now, and lack of consideration of a longer time horizon of years or even decades of life.
But in this blogpost I want to talk about “young PMR”: when people develop PMR in what should be the prime of their lives. By this I mean people under 65, and even people under 50. Nature does not respect round numbers: in my opinion, a disease that exactly resembles what we call PMR must surely exist in a few patients under 50, even if the creators of classification criteria consider it unwise to recruit those patients into phase 3 clinical trials. If it exactly resembles PMR, and all mimics have been excluded, what else are we to call it? But, just so that we can move on to discuss the challenges of treating patients with “young PMR”, let’s think about a hypothetical patient who has just turned 50 and has PMR. Definite PMR. PET-CT confirms it. All mimics excluded. These patients exist, and they need a very careful approach to treatment.
Treating “young PMR” can be a very different proposition to treating someone in their seventies or eighties. It’s worth taking time to think this through a biopsychosocial framework.
From a biological perspective, we expect a 50-year-old to live a long while yet. PMR does not, according to most evidence, shorten lifespan. So preventative medicine is crucial in these patients. Cardiovascular health, bone health, dental health and indeed mental health are just a few of the long-term considerations.
Do patients with “young PMR” have a worse prognosis? There may be some selection bias in our data: a greater tendency to refer younger patients with PMR to specialist centres, perhaps coupled with a reluctance to diagnose a younger patient with a condition treated with long-term glucocorticoids. From experience with other rheumatic diseases, we might wonder whether patients who have younger-onset immune-mediated inflammatory diseases might have a more severe course because susceptibility often correlates with severity: those with a greater number of “hits” from genetic and/or environmental susceptibility factors are likely to have both earlier-onset and more severe disease. We might also wonder whether delayed diagnosis, or difficulty accessing care due to difficulty fitting into diagnostic categories, might also cause sustained inflammation that is then more difficult to treat.
Psychologically, PMR can hit very hard, especially as the onset is so often subacute so patients do not have time to adapt to the new illness. Some people who develop PMR in their prime have never been seriously ill before. Acceptance that one has a chronic illness may be hard, especially if the validity of your diagnosis is frequently challenged.
Socially, one’s middle years are a time when life expects a lot from you, and you expect a lot from life. Many people are juggling multiple activities and responsibilities; work, household, caring for others, sport, socialising. People aged 50 may be just hitting their peak earning potential and early retirement of even a few years can have lifelong financial impacts during the decades afterwards. Although as physicians we are not qualified to offer financial advice, it is important to understand all the factors that patients must weigh up when making decisions about their health. If staying in work is important to a patient, then this needs to be discussed in the consultation if relevant to treatment considerations. Furthermore, younger patients are often more willing to challenge their physician, to do their own research and to have their own ideas. Health services designed around the “average” older patient with PMR may well not suit them. They may appear more confident and in control, and be more willing to take on the work of self-management, but they need support just as much as older patients do.
This was brought home to me by a study carried out by Dr Charikleia Chatizgeorgiou when she was undertaking her PhD at Leeds. You can read her study in preprint. The study looked at common comorbidities in those with PMR and giant cell arteritis using UK Biobank data. UK Biobank is a large-scale biomedical database and research resource that can be accessed globally by approved researchers, to enable scientific discoveries that improve human health. She conducted a matched case-control study describing the baseline characteristics of participants who reported they had ever received a diagnosis of PMR. Because of the inclusion criteria of UK Biobank (age between 40 and 69 and living in the UK), these participants were all individuals who developed PMR younger than average.
The findings of the study which most struck me was that UK Biobank participants who had been diagnosed with PMR had a significant self-reported health impact, compared to age-, sex-and geographically-matched participants who had never been diagnosed with PMR. Importantly, those no longer taking glucocorticoids at the time of the assessment visit still showed evidence of lasting mental health impact compared to controls. Participants with a history of PMR were also more likely to report hypertension and cataract than controls, but the impact of PMR showed up most of all in simple patient-reported health indicators, which are not usually collected systematically in medical records. Participants with PMR were more likely to self-report poor general health, and to have experienced at least several days of low mood over the two weeks prior to the assessment visit.
I thought again about these findings during our PMR Voices 2021 project, in which some people diagnosed with PMR expressed profound feelings of abandonment – “left to get on with it”. Some people diagnosed at a younger age said that they had made a shared decision with their doctor to stop long-term steroid therapy in view of the potential long-term health impacts and the many decades of life ahead of them, but they were left with no other treatment options and consequently had to live with ongoing pain and stiffness from PMR.
In my opinion, every person diagnosed with PMR before the national age of retirement –currently 66 in the UK - should be offered a specialist referral. Because when PMR strikes young, it strikes hard. If we as rheumatologists cannot be there for younger people with PMR, what are we even doing?
This blog is a personal account of what being involved in this research meant to me as a clinician, and what I have learned, and does not not necessarily reflect the view of my employer or the research funder. As well as thanking the co-authors of the cited paper, including Dr Chatzigeorgiou, Prof Ann Morgan, Prof Jenny Barrett, and Mr Eoin O’Sullivan, I would like to thank the charity Fight for Sight for part-funding this study. The cited research was conducted using data from UK Biobank www.ukbiobank.ac.uk, a major biomedical database, project ID: 5237.