Polymyalgia Research Articles

Characteristics of emerging new autoimmune diseases after COVID-19 vaccination: A sub-study by the COVAD group.

Despite the overall safety and efficacy of COVID-19 vaccinations, rare cases of systemic autoimmune diseases (SAIDs) have been reported post-vaccination. This study used a global survey to analyze SAIDs in susceptible individuals' post-vaccination. A cross-sectional study was conducted among participants with self-reported new-onset SAIDs using the COVID-19 Vaccination in Autoimmune Diseases (COVAD) 2 study dataset-a validated, patient-reported e-survey-to analyze the long-term safety of COVID-19 vaccines. Baseline characteristics of patients with new-onset SAIDs and vaccinated healthy controls (HCs) were compared after propensity score matching based on age and sex in a 1:4 ratio. Of 16 750 individuals, 74 (median age 52 years, 79.9% females, and 76.7% Caucasians) had new-onset SAID post-vaccination, mainly idiopathic inflammatory myopathies (IIMs) (n = 23, 31.51%), arthritis (n = 15; 20.53%), and polymyalgia rheumatica (PMR) (n = 12, 16.40%). Higher odds of new-onset SAIDs were noted among Caucasians (OR = 5.3; 95% CI = 2.9-9.7; p < .001) and Moderna vaccine recipients (OR = 2.7; 95% CI = 1.3-5.3; p = .004). New-onset SAIDs were associated with AID multimorbidity (OR = 1.4; 95% CI = 1.1-1.7; p < .001), mental health disorders (OR = 1.6; 95% CI = 1.3-1.9; p < .001), and mixed race (OR = 2.2; 95% CI = 1.2-4.2; p = .010), where those aged >60 years (OR = 0.6; 95% CI = 0.4-0.8; p = .007) and from high/medium human development index (HDI) countries (compared to very high HDI) reported fewer events than HCs. This study reports a low occurrence of new-onset SAIDs following COVID-19 vaccination, primarily IIMs, PMR, and inflammatory arthritis. Identified risk factors included pre-existing AID multimorbidity, mental health diseases, and mixed race. Revaccination was well tolerated by most patients; therefore, we recommend continuing COVID-19 vaccination in the general population. However, long-term studies are needed to understand the autoimmune phenomena arising post-vaccination.

Naïve Inflammatory Proteome Profiles of Glucocorticoid Responsive Polymyalgia Rheumatica and Rheumatic Arthritis Patients-Links to Triggers and Proteomic Manifestations.

Polymyalgia rheumatica (PMR) is an inflammatory disorder of unknown etiology, sharing symptoms with giant cell arthritis (GCA) and rheumatoid arthritis (RA). The pathogenic inflammatory roots are still not well understood, and there is a lack of extensive biomarker studies to explain the disease debut and post-acute phase. This study aimed to deeply analyze the serum proteome and inflammatory response of PMR patients before and after glucocorticoid treatment. We included treatment-naïve PMR patients, collecting samples before and after 3 months of treatment. For comparison, disease-modifying antirheumatic drug (DMARD)-naïve RA patients were included and matched to healthy controls (CTL). The serum proteome was examined using label-free quantitative mass spectrometry, while inflammation levels were assessed using multiplex inflammatory cytokine and cell-free DNA assays. The serum proteomes of the four groups comprised acute phase reactants, coagulation factors, complement proteins, immunoglobulins, and apolipoproteins. Serum amyloid A (SAA1) was significantly reduced by active PMR treatment. Cell-free DNA levels in PMR and RA groups were significantly higher than in healthy controls due to acute inflammation. Complement factors had minimal changes post-treatment. The individual serum proteome in PMR patients showed over 100 abundantly variable proteins, emphasizing the systemic impact of PMR disease debut and the effect of treatment. Interleukin (IL)-6 and interferon-gamma (IFN-γ) were significantly impacted by glucocorticoid treatment. Our study defines the PMR serum proteome during glucocorticoid treatment and highlights the role of SAA1, IL-6, and IFN-γ in treatment responses. An involvement of PGLYRP2 in acute PMR could indicate a response to bacterial infection, highlighting its role in the acute phase of the immune response. The results suggest that PMR may be an aberrant response to a bacterial infection with an exacerbated IL-6 and acute phase inflammatory response and molecular attempts to limit the inflammation.

P188 Frailty and its impact on patient-reported outcomes in polymyalgia rheumatica

Abstract Background/Aims Frailty is an increasingly important construct in the field of rheumatology, aiding the identification of individuals with increased vulnerability to accelerated clinical decline, and overall worse disease outcomes. The aim of this research was to explore the prevalence of frailty and its potential associated impact on patient reported outcomes (PROs) in a cohort of patients with polymyalgia rheumatica (PMR). Methods Patients with a diagnosis of PMR (fulfilling the 2012 EULAR/ACR Provisional Classification Criteria), who were in clinical remission and on active treatment with glucocorticoids were recruited from two centres. Patients were &amp;gt;3 months and &amp;lt;12 months from diagnosis. Frailty was defined by the 5 criteria of the widely validated Fried Phenotypic Frailty Index. Patient-reported outcomes included anxiety, using the Generalised Anxiety Disorder Assessment (GAD-7), mood using the Patient Health Questionnaire (PHQ-9), fatigue using the Functional Assessment of Chronic Illness Therapy-Fatigue Scale (FACIT-F), pain using the visual analogue scale (VAS) and overall health related quality of life using the Health Assessment Questionnaire-Disability Index (HAQ-DI). The associations between categorical variables were compared using the -test or Fishers exact test. The association between continuous variables and categorical variables was assessed using the Kruskal-Wallis test. Correlations were calculated using Pearson’s r. All analyses were conducted using R (R Core Team, 2022). A p-value of &amp;lt; 0.05 was considered as statistically significant. Results 51 consecutive patients were recruited, of which 56.9% (n = 29) were female. Using the Fried Phenotypic Index, 64.7% (n = 33) were classified as robust, 27.5% (n = 14) as pre-frail and 7.8% (n = 4) as frail. All patients classified as frail were female and had a statistically significantly higher BMI (p = 0.040) than those in the robust and pre-frail categories. Compared to robust individuals, those who were frail had statistically significant higher median GAD-7 (p = 0.003), PHQ-9 (p = 0.001), VAS (p = &amp;lt;0.001) and HAQ-DI (p = &amp;lt;0.001) scores. FACIT-F scores were also worse in those who were frail versus robust (p = 0.001). Conclusion Over one third of patients with PMR in this cohort were classified as pre-frail or frail. Increased frailty status was significantly correlated with worse PROs, including mood, pain, fatigue and overall quality of life. As frailty is a potentially reversible state, accurately identifying frailty, and implementing appropriate interventions is of utmost importance to ensure improved clinical outcomes in those with PMR. Disclosure P. Harkins: Grants/research support; British Society for Rheumatology. S. Cowley: None. R. Harrington: None. D. Kane: None. R. Conway: None.

P182 Exercise and activity in polymyalgia rheumatica: co-creating an education resource acceptable to patients and health professionals

Abstract Background/Aims People with PMR report experiencing pain, stiffness and weakness, often impacting everyday tasks. Many fear exercise will exacerbate symptoms and need reassurance that exercise is helpful and not harmful. However, no PMR-specific, exercise educational resource currently exists. The aim of this funded study was to co-create an exercise educational resource for, and with, people with PMR that would be acceptable to both patients and health professionals. Methods A steering group of people with PMR and UK clinicians/academics from Keele and Leeds universities was identified. People with PMR and clinical/academic health professionals with specific interest in PMR were invited to participate in a one-hour online focus group (FG), recruited via social media, the PMRGCAuk charity and professional networks. Ethical approval was obtained. Separate FGs (four with patient participants and two with multi-disciplinary health professionals), held during the summer of 2023, were facilitated by steering group members over two rounds. Exemplars of non-PMR health education literature were shared with participants in round 1 to prompt discussion and invite participant opinions about what might be essential exercise content and the optimal format of the new PMR educational resource. Summaries of proposed content and resource format suggestions were collated after each FG and informed the next iteration of the exercise and physical activity educational resource. In the final round 3 a prototype version of the educational booklet was shared with all FG participants via email. All participants were invited to provide final feedback and comments, after which the booklet was finalised. Results Participants desired a visually appealing pocket-size (A5), multiple-page, patient education booklet suitable for either printing or reading online. Colour-themed text and images with logos of PMRGCAuk and contributing universities were requested. Positive messaging promoting movement and physical activity, as well as exercise, were considered important. Feedback following draft booklet iterations concluded “a self-help guide” title was valuable, as were starter and progression instructions to accompany exercise illustrations. Participants desired explanation of prioritised “top tips”, including keeping moving, listening to your body, balancing rest and activity, and the physical and mental health benefits of exercising. Patient quotations were included with permissions together with signposting to other resources. Conclusion An exercise-focused education booklet, acceptable to both people with PMR and UK expert health professionals, has been co-created and professionally designed. Future research plans include piloting the feasibility of using this resource to support people living with PMR to engage and continue with exercise, and to evaluate the impact of people with PMR exercising. Disclosure A.V. O’Brien: Grants/research support; This project was funded by Polymyalgia Rheumatica, Giant Cell Arteritis UK charity - PMRGCAuk. S. Eng: None. S.L. Mackie: Consultancies; Roche/Chugai, Sanofi, AbbVie, AstraZeneca, Pfizer. Grants/research support; chief investigator on STERLING-PMR trial, funded by NIHR; patron of the charity PMRGCAuk. NIHR Leeds Biomedical Research Centre. Investigator on clinical trials for Sanofi, GSK, Sparrow. N. Scrafton: None. S. Hider: None. A. Tan: None. C. McClean: None. J. Barber: None. J. Gundle: None. M. Lonsdale: None. M. Baxter: None.

P035 BSR Digital resources- Spotlights and Podcasts The Story So Far

Abstract Background/Aims The British Society for Rheumatology (BSR) has an eLearning site for members. Due to increasing demand for online learning triggered in part by the COVID pandemic, a decision was made to create a BSR digital learning board and produce a broader range of content. Methods The Digital Learning Board (DLB) was formed in February 2021 consisting of trainees, nurses, Consultant Rheumatologists and Allied Healthcare Professionals from all around the 4 nations. After consultation and discussion, a decision was made to produce two main new formats of resources in addition to the online webinar series that was already established. This included Spotlights where a variety of blended learning resources including webinars, podcasts, eLearning cases and links to relevant journal articles are collated on a topic. The Talking Rheumatology Spotlight podcast series was also developed and shared on streaming platforms such as Spotify, iTunes and the eLearning website. This includes overview podcasts on topics as well as bonus case based episodes. Resources for the wider community (not limited to members) include the podcasts and ‘How to’ resources created for doctors and nurses starting work in the specialty for the first time. Numbers of podcast downloads, views on the eLearning website and quantitative and qualitative feedback is collected via eLearning website. Results The DLB have produced 30 spotlights with the most popular topics being axial spondyloarthropathy, psoriatic arthritis and Sjogren’s. The average rating for this series is 4.5/5. Examples of feedback; “all areas of great interest and very educational”“I really enjoyed the course, the fact that BSR included several podcasts allowed me to complete the course while doing my exercise. Great course really useful for my clinical practice” 28 podcasts have also been produced so far. There have been 15,500 downloads with significant international interest particularly in the US, Europe and Oceania. The most popular episodes so far have been Polymyalgia rheumatica and Sjogren's syndrome. The how to resources for trainees resource has been viewed 3,897 times by 1502 unique users, and the how to for nurses resource has been viewed 3159 times by 1360 unique .Examples of feedback from nurses “I have now been in post for 6 months and have learnt so much with this resource”“Great introductory course to Rheumatology” Conclusion Work is underway to promote the resources, seek feedback and suggestions for further topics. Many face to face education programmes including the BSR’s own are running again but research (1) and behaviour suggest blended learning is here to stay and complements in person learning well. We are exploring this area further through the development of pre course learning for our face-to-face courses. Disclosure R. Benson: None. L. Coulson: Other; Louise works for the BSR as Digital Events Manager. W.J. Gregory: Honoraria; W.G. has received honoraria for speaker and advisory board from Abbive, Novartis, Pfizer and UCB.. J. Lemon: None. Y.L. Man: None. K. Maxey: Other; Kate works for the BSR as events and education officer. D. Pilbury: None. M. Serrano: Other; Marina works for the BSR as acting Head of Education. C. Wincup: None. P.A. Watson: Other; I am employed by the British Society for Rheumatology as Digital Learning Editor (2PA per week). S. Ellis: None. E. Chibueze Mbanasor: None. M. Malik: None. B. Lakshmana Murthy: None.

P187 Fatigue in people with polymyalgia rheumatica: a key unmet need?

Abstract Background/Aims Polymyalgia rheumatica (PMR) is common in older adults, causing severe pain and stiffness, particularly in the shoulder and hip girdles. Glucocorticoids treatment generally focusses on reducing pain and stiffness, but there are other aspects to quality of life. Fatigue is a well-recognised symptom in other inflammatory rheumatological conditions and has been highlighted by people with PMR as a neglected symptom. Methods People with newly diagnosed PMR in English general practice (2012-2014, n = 652) were recruited to a postally-administered cohort study. Surveys included items relating to PMR, general health and well-being. The FACIT-Fatigue questionnaire was included in the survey at diagnosis, 1, 12, 24 and (mean) 61 months follow-ups. Latent class growth analysis was used to define trajectories, based on FACIT-Fatigue scores over time. Trajectories were characterised and compared at baseline and over time. Results 244 people participated at 24 months and 197 at 61 months. Individuals were allocated to one of five fatigue trajectories. Each represented a different average level of fatigue at diagnosis that remained stable over time (Table). From least to most fatigued, trajectory sizes were 111, 122, 211, 133 and 75. Two thirds of the cohort (the three “worst” trajectories) were, on average, more fatigued than reported in the general population. Worse fatigue trajectory was associated with female sex, higher pain and stiffness, higher levels of anxiety, depression, insomnia, and poorer physical function at baseline and throughout. Conclusion Fatigue is common in inflammatory conditions, but under-recognised in PMR. Fatigue severity at the time of diagnosis appears indicative of fatigue levels up to five years later and is associated with a broad range of health constructs at diagnosis and later. This represents an unmet need in a sizable proportion of those with PMR, who may need more help to manage their condition and allow them to return to full function. These findings also present a potential avenue for early identification of those with a poor prognosis related to their PMR. Disclosure S. Muller: None. J. Belcher: None. S. Hider: None. T. Helliwell: None. C.D. Mallen: None.

E008 Review of rheumatology care for women of childbearing age

Abstract Background/Aims Autoimmune rheumatic diseases are frequently diagnosed in women of childbearing age. Managing these conditions during pregnancy is complex because commonly used disease-modifying antirheumatic drugs (DMARDs) can pose risks. Specialized care for this patient group is crucial, necessitating adherence to established rheumatology practice standards and British Society for Rheumatology (BSR) guidelines. Our main objective is to review management of childbearing age females with rheumatic diseases attending Basildon hospital as per BSR guidelines. Methods The data for this retrospective study was collected from the electronic records of female patients attending the rheumatology outpatient clinic at Basildon Hospital between September and December 2022. We reviewed age, the specific rheumatological diagnosis, disease duration, disease activity, current medications, and whether general advice or pregnancy-specific advice was provided. Results Out of the initial cohort of 63 female patients, 48 (76%) aged between 18-55 years were selected as women of childbearing age, The median age was 46 years, with 65% in the age groups of 31-50 years, 25% in group 51-55 years and 10% 18-30 years age group. 58% of patients had inflammatory arthritis, 18.7% had connective tissue disease, 18.7% had other conditions such as hypermobility, osteoarthritis, and regional pain syndrome, 2.3% were diagnosed with vasculitis and 2.3% with polymyalgia rheumatica. Breakdown of inflammatory arthritis patients revealed rheumatoid arthritis in 64%, psoriatic arthritis 15% and ankylosing spondylitis 12%. Axial spondyloarthropathy, palindromic rheumatism, and juvenile idiopathic arthritis, each accounted for 3%. Regarding prescribed medications, 66% of the individuals were on conventional DMARDs, biologics 24%, corticosteroids 6% and 4% were on other treatments. The DMARDs prescribed were methotrexate 27%, hydroxychloroquine 19%, sulfasalazine 13%, mycophenolate 3% and leflunomide 1%. The majority of patients had disease activity in remission or mild, each accounting for 23%. Moderate and severe disease activity were reported in 21% each of the audited sample. Patients received advice specifically related to pregnancy 29%, while 71% did not have documented pregnancy-related guidance. 70% of females of reproductive age received general advice. Conclusion The study highlights a gap in pregnancy-related advice among the childbearing age patients as only 29% received guidance on pregnancy. This underpins the need for proactive counselling and education for female patients in routine practice, work in partnership with obstetric teams, and primary care to reduce potential pregnancy related complications and improve outcomes. Disclosure M. Mohareb: None. S. Ahmed: None. A. Bharadwaj: None. A. Nandagudi: None.

P041 Health-related quality of life in patients receiving glucocorticoids for a rheumatic disease and associated factors : a cross-sectional study

Abstract Background/Aims Glucocorticoids (GCs) used in the treatment of inflammatory rheumatic conditions can impact on patients in different ways. The objective of this study was to identify socio-demographic and clinical factors associated with health-related quality of life (HRQoL) in patients undertaking GCs for a rheumatic disease. Methods This was a cross-sectional study using online survey methods. Participants taking GCs for a rheumatic disease from Australia, United Kingdom and the United States of America were included. HRQoL was measured using the EQ-5D-5L converted into a linear EQ5D index value. Eight explanatory factors (age, sex, country, educational level, employment status, disease group, self-reported disease state, and dose of GCs) were tested for potential association with HRQoL using univariable and multivariable (hierarchical) analyses. Factors found to have a significant association with EQ5D index at a P &amp;lt; 0.05 level in the multivariable model were considered to be independently associated with HRQoL. Results A total of 945 patients completed the EQ-5D-5L with no missing values: UK n = 742 (79%); USA n = 139 (15%); Australia/New Zealand n = 64 (7%); mean age 57.6 (SD = 13.6); 833 (88%) women. Participants with inflammatory arthritis n = 197 (21%), connective tissue disease and/or vasculitis n = 402 (43%), giant cell arteritis and/or polymyalgia rheumatica n = 346 (37%) were included. Those with self-reported active disease were 726 (77%). Mean (SD) EQ5D index was 0.64 (0.25); there were significant differences between groups in EQ-5D-5L index by age (median split, t=-3.77, p &amp;lt; 0.001); sex (t = 3.40, p &amp;lt; 001); educational level (F[3]=10.51, p &amp;lt; 0.001); employment status (F[6]=37.67, P &amp;lt; 0.001); disease group (T[2]=20.23, p &amp;lt; 0.001); disease state (T=-8.46, p &amp;lt; 0.001); and the dose of GCs(T = 2.95, p &amp;lt; 0.001). Worse HRQoL was associated with older age, being female, low educational attainment, being unemployed, having an inflammatory arthritis or a CTD, disease being active, and taking a higher dose of GC. Of the eight factors that were tested in the multivariable model, five were found to be independently associated with HRQoL. These were: disease state (β = 0.12, p &amp;lt; 0.001), disease group (β = 0.06, p &amp;lt; 0.001), employment status (β = 0.05, p &amp;lt; 0.001), sex (β = 0.05, p &amp;lt; 0.001), and educational level (β = 0.04, p &amp;lt; 0.001). This 5-factor model explained 18% of the variance in EQ5D index scores (F[1, 926] = 4.97, P = 0.026). The excluded factors in the model were age, dose, and country. Conclusion In our data, we identified 5 factors independently associated with worse HRQoL in patients being treated with GCs. Over 80% of the variance was not explained by our model likely due to use of a generic HRQoL measure (EQ-5D-5L) as outcome variable, and the small number of explanatory variables tested within this survey. Further research is required to identify potential targets to support interventions. Disclosure S. Janagan: None. K. Lim: None. R. Bhogal: None. S. Bridgewater: Grants/research support; Vifor pharma. C. Silverthorne: None. P. Richards: None. J. Dawson: None. E. Dures: Grants/research support; Vifor pharma. C. Hill: None. S. Goodman: Consultancies; UCB. Grants/research support; Novartis. S.L. Mackie: Consultancies; Consultancy on behalf of her institution for Roche/Chugai, Sanofi, AbbVie, AstraZeneca, Pfizer. Other; Investigator on clinical trials for Sanofi, GSK, Sparrow, speaking/lecturing on behalf of her institution for Roche/Chugai, Vifor, Pfizer, UCB, Novartis and AbbVie, ; chief investigator on STERLING-PMR trial, funded by NIHR; patron of the charity PMRGCAuk, No personal remuneration was received for any of the above activities. Support from Roche/Chugai to attend EULAR2019 in person and from Pfizer to attend ACR Convergence 2021 virtually., SLM is supported in part by the NIHR Leeds Biomedical Research Centre., The views expressed in this article are those of the authors and not necessarily those of the NIHR,the NIHR Leeds Biomedical Research Centre, the National Health Service or the UK Department of Health. M. Ndosi: Grants/research support; Vifor pharma. J.C. Robson: Consultancies; Vifor Pharma. Member of speakers’ bureau; Vifor Pharma. Grants/research support; Unrestricted Grant/research support from: Vifor Pharma.

P185 Anatomical patterns of pain in primary care patients with polymyalgia rheumatica compared to the general population

Abstract Background/Aims Polymyalgia rheumatica (PMR) is common in older adults, causing severe pain and stiffness, particularly in the shoulder and hip girdles. A tapered course of glucocorticoids is recommended, but not always straightforward, with frequent symptom recurrence. Reasons are unclear, but partial masking of painful comorbidities (e.g., osteoarthritis) may play a part. Methods 652 people with incident PMR were recruited from English general practice (2012-2014) to a postal survey study (questionnaires at diagnosis, 1, 4, 8, 12, 18, 24, [mean] 61 months). Surveys included a body manikin (front and back views). Participants were asked to shade anywhere they experienced pain &amp;gt;1 day in the last month. 44 mutually exclusive areas were defined as painful or not. For comparison, an age-gender matched random sample of 652 people was selected from a general population survey in North Staffordshire that used the same manikins. Pain prevalence was compared between the 2 studies. Results In the PMR study, surveys were completed by 244 participants at 24 months and 197 at 61 months. Pain was reported in a mean of 17.1 areas at diagnosis (Table). Bilateral shoulder and hip pain were present in the majority at diagnosis (shoulder 81%; hips 59%) and in around a third and a fifth of patients respectively throughout the study. The prevalence of bilateral shoulder and hip pain remained higher than in the general population sample, while unilateral pain in these areas was less common. Bilateral hand (27%) and knee (45%) pain were also common at diagnosis. After 1 month, the mean number of pain areas was 7 - similar to the general population. However, prevalence of bilateral shoulder and hip pain remained higher than the general population throughout follow-up. Unilateral hip and knee pain were persistently less common in those with PMR, whilst unilateral hip pain was more common. Conclusion Bilateral pain was reported more commonly across the disease course in people with PMR than the general population. Causes of pain cannot be accurately attributed, but are likely multifactorial, including PMR disease activity, treatment and painful comorbidities. However, the persistence of bilateral pain suggests untreated PMR symptoms, requiring further research. Disclosure N. Hammad: None. R. Partington: None. S. Hider: None. H. Butt: None. T. Helliwell: None. C. Mallen: None. S. Muller: None.

P191 Giant cell arteritis relapses occur in a different arterial territory as compared to diagnosis

Abstract Background/Aims Ultrasonography (US) for diagnosis of large vessel vasculitis (LVV) is a recommended first line diagnostic procedure. We have been running an US led diagnostic pathway in our centre since January 2017. This pathway is also used to diagnose relapsing disease. We present US defined anatomical distribution of relapses in consecutive patients. Methods Patients in whom new and relapsing disease was diagnosed using US were included. Protocolised US examination of the common carotid system (segments of common carotid, external carotid and branches) and those of the subclavian system (segments from origin of subclavian to end of axillary artery and branches) were carried out at diagnosis and relapse. Halo sign in at least two different arteries was needed for establishing new or relapsing disease. Disease characterisation was classified as being in the carotid system, subclavian system, or both. The nature of the disease at relapse was compared to that at diagnosis. Results 279 patients were diagnosed with LVV using US since Jan 2017. 57 (20%) were diagnosed as having a relapse using US. 43/57 (75%) and 28/57 (49%) had involvement of the carotid system at baseline and relapse respectively. 22/57 (39%) and 44/57 (77%) had involvement of the subclavian system at baseline and relapse respectively. A comparison of the disease involvement is as in Figure 1. Conclusion The branches of the common carotid artery (external carotid artery) are commonly involved at diagnosis, but relapse occurs more frequently in the subclavian artery branches. This is especially true when disease affects the carotid system at baseline. The presentation of patients with subclavian system disease is often with only constitutional symptoms and with shoulder girdle pain and stiffness. This may be mistaken for being a polymyalgia rheumatica relapse. It is essential to examine the subclavian system of arteries in patients with LVV before diagnosing polymyalgia rheumatic relapse. Disclosure S. Alanoor: None. G. Ducker: None. C.B. Mukhtyar: None.

Rheumatology: What You May Have Missed in 2023.

Many patients with rheumatologic conditions receive care from physicians other than rheumatologists. Here we note key findings from 6 studies in rheumatology published in 2023 that offer valuable insights for internal medicine specialists and subspecialists outside of rheumatology. The first study investigated the effect of low-dose glucocorticoids on patients with rheumatoid arthritis (RA) over 2 years and challenged existing perceptions about the risks of glucocorticoids in this setting. The second study focused on the updated guideline for preventing and treating glucocorticoid-induced osteoporosis. With the chronic and widespread use of glucocorticoids, the American College of Rheumatology emphasized the importance of assessing fracture risk and initiating pharmacologic therapy when appropriate. The third study explored the potential use of methotrexate in treating inflammatory hand osteoarthritis, suggesting a novel approach to managing this challenging and common condition. The results of the fourth article we highlight suggest that sarilumab has promise as an adjunct treatment of polymyalgia rheumatica relapse during glucocorticoid dosage tapering. The fifth study evaluated sublingual cyclobenzaprine for fibromyalgia treatment, noting both potential benefits and risks. Finally, the sixth article is a systematic review and meta-analysis that assessed the therapeutic equivalence of biosimilars and reference biologics in the treatment of patients with RA. Knowledge of this recent literature will be useful to clinicians regardless of specialty who care for patients with these commonly encountered conditions.

Development and organization of Tabriz University of Medical Sciences Vasculitis Registry: TUOMS-VR

This manuscript describes the establishment and initial results of a patient-driven registry in Tabriz, Iran to collect demographic, clinical, and paraclinical data, treatment and outcomes of patients with vasculitis. The Tabriz University of Medical Sciences Vasculitis Registry (TUOMS-VR), a patient-driven, prospective and web-based disease registry system, is conducted at the Connective Tissue Diseases Research Center, TUOMS located in Tabriz, Iran. The primary objective of this registry is to comprehensively document prospective data of patients diagnosed with systemic or single-organ vasculitis according to the Chapel Hill Consensus Conference nomenclature and definitions. To date, the registry has successfully enrolled 743 patients. The gender distribution within this existing cohort is balanced, with 50.5% male and 49.5% female participants. The most frequently diagnosed condition was Behcet’s disease, representing 56.5% of cases. Polymyalgia rheumatica also emerged as a notable diagnosis, accounting for 13.7% of cases. By providing a comprehensive and prospective documentation of vasculitis cases, the TUOMS-VR aims to enhance vasculitis patients care and outcomes.

Recommendations of the French Society of Rheumatology for the management in current practice of patients with polymyalgia rheumatica

ObjectiveTo develop recommendations for the routine management of patients with polymyalgia rheumatica (PMR). MethodsFollowing standard procedures, a systematic review of the literature by five supervised junior rheumatologists, based on the questions selected by the steering committee (5 senior rheumatologists), was used as the basis for working meetings, followed by a one-day plenary meeting with the working group (15 members), leading to the development of the wording and determination of the strength of the recommendations and the level of agreement of the experts. ResultsFive general principles and 19 recommendations were drawn up. Three recommendations relate to diagnosis and the use of imaging, and five to the assessment of the disease, its activity and comorbidities. Non-pharmacological therapies are the subject of one recommendation. Three recommendations concern initial treatment based on general corticosteroid therapy, five concern the reduction of corticosteroid therapy and follow-up, and two concern corticosteroid dependence and steroid-sparing treatments (anti-IL-6). ConclusionThese recommendations take account of current data on PMR, with the aim of reducing exposure to corticosteroid therapy and its side effects in a fragile population. They are intended to be practical, to help practitioners in the day-to-day management of patients with PMR.

A Cautionary Note on Pembrolizumab use in Patients with Ascending Aortic Aneurysms.

We describe a case of a patient treated with pembrolizumab (an immune checkpoint inhibitor) for metastatic scalp melanoma. He had a previous history of colorectal cancer, prostatic cancer and chronic polymyalgia rheumatica. The patient was known to have a stable ascending aortic aneurysm of 4.5 cm. However, he developed a rapid expansion of the ascending aortic aneurysm with the size crossing the threshold for surgery. The patient was referred to the cardiothoracic surgery service for intervention and he subsequently underwent surgery. The patient was electively admitted one week later for resection of aortic aneurysm, aortoplasty and external graft fixation. Pathologically, gross evidence of dissection was not identified; however, the histological analysis of the media showed laminar medial necrosis, multifocal in nature, with occasional clusters of histiocytic cells appreciated at their edge reminiscent of that seen in an inflammatory aortitis (granulomatous/giant cell type). Immune checkpoint inhibitor-induced aortitis is becoming increasingly evident, and its presentation can vary. It has been discovered incidentally on surveillance imaging with the use of nivolumab. In other cases, patients have been symptomatic to severely symptomatic. Atezolizumab with carboplatin and etoposide has been reported to cause abdominal aortitis which was responsive to corticosteroids and subsequent discontinuation of atezolizumab. Pembrolizumab has been linked to a case of transverse aortic arch aortitis. In our case, the inflammatory aortitis due to pembrolizumab was the cause of the rapid expansion of the ascending aortic aneurysm. Patients with known aortic aneurysms should undergo careful surveillance when commencing immune-checkpoint inhibitor therapy. Immune checkpoint inhibitors are being increasingly used in the treatment of metastatic malignancy. However, they are a relatively new group of medications, and the side effect profile of each is yet to be fully recognised. Aortitis has occurred with several different immune checkpoint inhibitors.Patients with known aortic aneurysms should undergo careful surveillance when commencing immune checkpoint inhibitors.All interventional therapeutic options should be considered early in these patients on the development of aneurysmal expansion.

The effect of prednisolone and a short-term prednisolone discontinuation for the diagnostic accuracy of FDG-PET/CT in polymyalgia rheumatica—a prospective study of 101 patients

Purpose2-[18F]Fluoro-2-deoxy-D-glucose (FDG)–positron emission tomography (PET)/computed tomography (CT) has been suggested as an imaging modality to diagnose polymyalgia rheumatica (PMR). However, the applicability of FDG-PET/CT remains unclear, especially following glucocorticoid administration. This study aimed to investigate the diagnostic accuracy of FDG-PET/CT before and during prednisolone treatment, as well as following short-term prednisolone discontinuation.MethodsTreatment naïve suspected PMR patients were clinically diagnosed at baseline and subsequently had an FDG-PET/CT performed. Patients diagnosed with PMR were administered prednisolone following the first FDG-PET/CT and had a second FDG-PET/CT performed after 8 weeks of treatment. Subsequently, prednisolone was tapered with short-term discontinuation at week 9 followed by a third FDG-PET/CT at week 10. An FDG-PET/CT classification of PMR/non-PMR was applied, utilizing both the validated Leuven score and a dichotomous PMR score. The final diagnosis was based on clinical follow-up after 1 year.ResultsA total of 68 and 27 patients received a final clinical diagnosis of PMR or non-PMR. A baseline FDG-PET/CT classified the patients as having PMR with a sensitivity/specificity of 86%/63% (Leuven score) and 82%/70% (dichotomous score). Comparing the subgroup of non-PMR with inflammatory diseases to the PMR group demonstrated a specificity of 39%/54% (Leuven/dichotomous score). After 8 weeks of prednisolone treatment, the sensitivity of FDG-PET/CT decreased to 36%/41% (Leuven/dichotomous score), while a short-term prednisolone discontinuation increased the sensitivity to 66%/60%.ConclusionFDG-PET/CT has limited diagnostic accuracy for differentiating PMR from other inflammatory diseases. If FDG-PET/CT is intended for diagnostic purposes, prednisolone should be discontinued to enhance diagnostic accuracy.Trial registrationClinicalTrials.gov (NCT04519580). Registered 17th of August 2020.

Mortality associated with polymyalgia rheumatica in the United States in the 1999-2020 period: a multiple-cause-of-death study.

Multicause-of-death methods were used to analyze mortality and leading causes of death associated with polymyalgia rheumatica (PMR) in the United States from 1999 to 2020. We analyzed mortality data from the Centers for Disease Control and Prevention (CDC) Data analysis system and selected death certificates that listed PMR as the cause of death based on the International Statistical Classification of Diseases and Related Health Problems (ICD-10) category code. Relevant mortality rates, number of deaths and historical trends were analyzed. The number of PMR-related deaths and age-standardized mortality rate (ASMR) trend charts were made using Excel 2010 version and trend lines were added. Over the last 22 years, the total number of PMR-related deaths in the United States was 15,421 women (89.8%), a ratio of about 1:9 men to women. When PMR is listed as the underlying cause of death, the ASMR for women and men (per 100,000 people) is approximately 1.8-5.1:1, and when it is listed as the non-underlying cause of death, it is 1.8-3.3:1. PMR deaths are more frequent in individuals aged 70 years and above, with patients aged 80 years and above being most affected. Among different ethnicities, the highest number of deaths was found in Caucasians, followed by Black or African American. When it comes to causes of death, heart disease still ranks first, followed by cancer. In addition, we also found that when PMR combined with malignant tumors as a multiple cause of death, the number of female deaths was higher than that of male deaths, the overall number of deaths of both showed an upward trend, and the overall ASMR of both showed a downward trend. In the past 22 years, we have observed a low mortality rate of PMR in the United States. However, for patients with PMR, especially elderly women, medical workers should be vigilant and pay attention to whether they are combined with other complications, such as malignant neoplasms, and make timely diagnosis and treatment to further reduce the mortality rate of patients with PMR.

Should all patients with polymyalgia rheumatica have a vascular ultrasound assessment?

There is a growing appreciation that both giant cell arteritis (GCA) and polymyalgia rheumatica (PMR) are closely interrelated conditions that have significant overlap in aetiology, clinical characteristics and treatment regimens....

Prevalence, characteristics, and outcome of subclinical vasculitis in polymyalgia rheumatica: a retrospective cohort study.

Two recent meta-analyses reported subclinical vasculitis in 22-23% of patients with polymyalgia rheumatica (PMR). We aimed to evaluate the prevalence, characteristics, and outcome of subclinical vasculitis among our PMR patients. Consecutive patients with GCA/PMR spectrum disease with isolated PMR symptoms who underwent FDG PET imaging between 2003-2020 and who were followed for ≥6 months, were included retrospectively. Vasculitis was defined as FDG uptake ≥ grade 2 in any vessel. We included 337 patients, of whom 31 (9%) with subclinical vasculitis. Among those with subclinical vasculitis, 21 (58%) had isolated large vessel vasculitis, 3 (10%) had isolated cranial vasculitis and 7 (23%) had both cranial and large vessel vasculitis. The glucocorticoid (GC) starting dose and GC doses during follow-up were higher in those with subclinical vasculitis until 12 months after diagnosis (p< 0.001). There was no difference in the duration of GC treatment (25 vs 20 months, p= 0.187). Cox proportional hazard regression analyses showed no difference in the proportion of patients able to stop GC (HR 0.78 [95% CI 0.49-1.25], p= 0.303) and in the proportion of patients with relapse (HR 0.82 [95%CI 0.50-1.36], p= 0.441). Only 9% of our PMR patients had subclinical vasculitis with a predilection for large vessel vasculitis. There were no differences in relapse rate and duration of GC treatment, however those with subclinical vasculitis received higher GC doses until 12 months after diagnosis. Prospective interventional trials are needed to evaluate the outcome of PMR patients with and without subclinical vasculitis treated with similar GC protocol.

Did the first description of patients with polymyalgia rheumatica take place in Scotland or in Denmark?

The first description of polymyalgia rheumatica (PMR) is generally attributed to Dr. Bruce. In an 1888 article entitled Senile rheumatic gout, he described five male patients aged from 60 to 74 years whom he had visited at the Strathpeffer spa in Scotland. In 1945, Dr. Holst and Dr. Johansen reported on five female patients examined over several months at the Medical Department of Roskilde County Hospital in Denmark. These patients suffered from hip, upper arms, and neck pain associated with elevated ESR and constitutional manifestations such as low-grade fever or loss of weight. In the same year, Meulengracht, another Danish physician, reported on two patients with shoulder pain and stiffness associated with fever, weight loss, and an increased erythrocyte sedimentation rate. As in the five patients reported by Dr. Holst and Dr. Johansen, a prolonged recovery time was recorded. On reading and comparing these three accounts, we question whether it is correct to attribute the first description of PMR to Dr. Bruce and put forward shifting this accolade to the three Danish physicians.

Atypical Systemic Lupus Erythematosus Presenting as Polymyalgia Rheumatica in an Elderly Native American Male

Atypical Systemic Lupus Erythematosus Presenting as Polymyalgia Rheumatica in an Elderly Native American Male