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. Subclinical GCA in PMR is becoming increasingly recognised, and there is evolving evidence that this may be a more aggressive disease phenotype than PMR. Ultrasound (US) lends itself well as a screening tool for GCA in PMR; it is inexpensive, non-invasive, widely available, lacks ionising radiation, may be performed at the bedside and is recommended by EULAR as a first-line investigation for suspected GCA. There is insufficient evidence to currently recommend that all patients with PMR should have a US assessment for vascular involvement. However, as clinical and laboratory parameters alone do not accurately diagnose patients with subclinical GCA, we suggest that vascular US will be increasingly performed by rheumatologists in practice to identify these patients with PMR, preferably as part of larger prospective outcome studies.

Subclinical giant cell arteritis increases the risk of relapse in polymyalgia rheumatica.

OBJECTIVE: The aim of the present study was to determine the clinical significance of subclinical giant cell arteritis (GCA) in polymyalgia rheumatica (PMR) and ascertain its optimal treatment approach. METHODS: Patients with PMR who fulfilled the 2012 European Alliance of Associations for Rheumatology/American College of Rheumatology Provisional Classification Criteria for PMR, did not have GCA symptoms and were routinely followed up for 2 years and were stratified into two groups, according to their ultrasound results: isolated PMR and PMR with subclinical GCA. The outcomes (relapses, glucocorticoid use and disease-modifying antirheumatic drug treatments) between groups were compared. RESULTS: We included 150 patients with PMR (50 with subclinical GCA) with a median (IQR) follow-up of 22 (20-24) months. Overall, 47 patients (31.3 %) had a relapse, 31 (62%) in the subclinical GCA group and 16 (16%) in the isolated PMR group (p<0.001). Among patients with subclinical GCA, no differences were found in the mean (SD) prednisone starting dosage between relapsed and non-relapsed patients (32.4±15.6 vs 35.5±12.1 mg, respectively, p=0.722). Patients with subclinical GCA who relapsed had a faster prednisone dose tapering in the first 3 months compared with the non-relapsed patients, with a mean dose at the third month of 10.0±5.2 versus 15.2±7.9 mg daily (p<0.001). No differences were found between relapsing and non-relapsed patients with subclinical GCA regarding age, sex, C reactive protein and erythrocyte sedimentation rate. CONCLUSIONS: Patients with PMR and subclinical GCA had a significantly higher number of relapses during a 2-year follow-up than patients with isolated PMR. Lower starting doses and rapid glucocorticoid tapering in the first 3 months emerged as risk factors for relapse.

Validation and incorporation of digital entheses into a preliminary GLobal OMERACT Ultrasound DActylitis Score (GLOUDAS) in psoriatic arthritis.

OBJECTIVES: The main objective was to generate a GLobal OMERACT Ultrasound DActylitis Score (GLOUDAS) in psoriatic arthritis and to test its reliability. To this end, we assessed the validity, feasibility and applicability of ultrasound assessment of finger entheses to incorporate them into the scoring system. METHODS: The study consisted of a stepwise process. First, in cadaveric specimens, we identified enthesis sites of the fingers by ultrasound and gross anatomy, and then verified presence of entheseal tissue in histological samples. We then selected the entheses to be incorporated into a dactylitis scoring system through a Delphi consensus process among international experts. Next, we established and defined the ultrasound components of dactylitis and their scoring systems using Delphi methodology. Finally, we tested the interobserver and intraobserver reliability of the consensus- based scoring systemin patients with psoriatic dactylitis. RESULTS: 32 entheses were identified in cadaveric fingers. The presence of entheseal tissues was confirmed in all cadaveric samples. Of these, following the consensus process, 12 entheses were selected for inclusion in GLOUDAS. Ultrasound components of GLOUDAS agreed on through the Delphi process were synovitis, tenosynovitis, enthesitis, subcutaneous tissue inflammation and periextensor tendon inflammation. The scoring system for each component was also agreed on. Interobserver reliability was fair to good (κ 0.39-0.71) and intraobserver reliability good to excellent (κ 0.80-0.88) for dactylitis components. Interobserver and intraobserver agreement for the total B-mode and Doppler mode scores (sum of the scores of the individual abnormalities) were excellent (interobserver intraclass correlation coefficient (ICC) 0.98 for B-mode and 0.99 for Doppler mode; intraobserver ICC 0.98 for both modes). CONCLUSIONS: We have produced a consensus-driven ultrasound dactylitis scoring system that has shown acceptable interobserver reliability and excellent intraobserver reliability. Through anatomical knowledge, small entheses of the fingers were identified and histologically validated.

Real-world outcomes of a dedicated fast-track polymyalgia rheumatica clinic.

OBJECTIVES: To examine the clinical impact of a fast-track PMR clinic to enable early diagnosis and treatment, and to define both patient and disease characteristics in newly diagnosed PMR. METHODS: Primary care physicians were invited to refer patients with new PMR to our fast-track clinic. Referral criteria included new onset shoulder or pelvic girdle pain and/or stiffness with elevated inflammatory markers in patients over 50 years. All patients were seen within 72 h of referral. Patients with a rheumatology diagnosis of PMR had an ultrasound (US) of their temporal and axillary arteries. RESULTS: 172 patients were referred from primary care over 12 months. 39% of patients referred with suspected PMR had an alternative diagnosis for which PMR regimen glucocorticoids was inappropriate. 55% of the non-PMR diagnoses were other inflammatory rheumatological conditions requiring follow-up. Only 20% of patients referred from primary care already on glucocorticoids were commenced on bone protection. PMR patients were co-morbid with a mean of 2.5 other conditions. 75% of PMR patients experienced a glucocorticoid-related adverse event in the first 12 months of treatment. 16% of patients with new PMR had ultrasound features of subclinical giant cell arteritis. CONCLUSION: The commencement of glucocorticoid therapy should be deferred until after specialist evaluation to enable an accurate clinical diagnosis. A delay in treatment can only realistically be avoided if GPs have access to a Fast-track PMR clinic. We believe that rheumatologists should consider establishing fast-track PMR clinics and this study provides a strong case for and a template to support this practice innovation.

Temporal trends in COVID-19 outcomes in people with rheumatic diseases in Ireland: data from the COVID-19 Global Rheumatology Alliance registry.

OBJECTIVES: Although evidence is accumulating globally, data on outcomes in rheumatic disease and COVID-19 in Ireland are limited. We used data from the COVID-19 Global Rheumatology Alliance (C19-GRA) to describe time-varying COVID-19 outcomes for people with rheumatic disease in Ireland. METHODS: Data entered into the C19-GRA provider registry from Ireland between 24 March 2020 and 9 July 2021 were analysed. Differences in the likelihood of hospitalization and mortality according to demographic and clinical variables were investigated using Chi-squared test or Fisher's exact test, as appropriate. Trends in odds of hospitalization and mortality over time were investigated using logistic regression with the time period as a categorical variable. RESULTS: Of 212 cases included, 59.4% were female and median age was 58.0 years (range 13-96). Of the 212 cases, 92 (43%) were hospitalized and 22 (10.4%) died. Increasing age, a diagnosis of gout, ever smoking, glucocorticoid use, having comorbidities and specific comorbidities of cancer, cardiovascular and pulmonary disease were more common in those hospitalized. A diagnosis of inflammatory arthritis, csDMARD and/or b/tsDMARD use were less frequent in those hospitalized. Increasing age, a diagnosis of gout, ever smoking, having comorbidities and specific comorbidities of obesity, cardiovascular and pulmonary disease were more common in those who died. Odds of hospitalization or mortality did not change over time. CONCLUSION: No temporal trend was observed in either COVID-19-related hospitalization or mortality outcomes for people with rheumatic disease in Ireland.

Online education in a hurry: Delivering pediatric graduate medical education during COVID-19.

In the setting of the coronavirus disease 2019 (COVID-19) pandemic, an emergency hospital-wide eWork policy was enacted at Boston Children's Hospital on March 16, 2020. The number of clinicians on campus was restricted to only essential personnel, guidelines limited clinical care delivery to solely non-elective patients, and strict maximums were placed on the numbers of people allowed to congregate in the same physical space. With this abrupt transition to social distancing and electronic communication, the established approach to educating graduate medical trainees became obsolete overnight. Anticipating significant impact on trainee and faculty professional and personal lives, the importance of adaptive teaching strategies was evident. This document details one approach to redesigning the clinical learning system including a description of the learners and environment, the pedagogical principles that guided the approach, and technological tools used in implementation. Additionally, available literature pertinent to this topic is explored, assessment of the work to date is presented, and suggestions are provided regarding future directions related to online graduate medical education.

EULAR/eumusc.net standards of care for rheumatoid arthritis: cross-sectional analyses of importance, level of implementation and care gaps experienced by patients and rheumatologists across 35 European countries.

OBJECTIVE: As part of European League against Rheumatism (EULAR)/European Musculoskeletal Conditions Surveillance and Information Network, 20 user-focused standards of care (SoCs) for rheumatoid arthritis (RA) addressing 16 domains of care were developed. This study aimed to explore gaps in implementation of these SoCs across Europe. METHODS: Two cross-sectional surveys on the importance, level of and barriers (patients only) to implementation of each SoC (0-10, 10 highest) were designed to be conducted among patients and rheumatologists in 50 European countries. Care gaps were calculated as the difference between the actual and maximum possible score for implementation (ie, 10) multiplied by the care importance score, resulting in care gaps (0-100, maximal gap). Factors associated with the problematic care gaps (ie, gap≥30 and importance≥6 and implementation<6) and strong barriers (≥6) were further analysed in multilevel logistic regression models. RESULTS: Overall, 26 and 31 countries provided data from 1873 patients and 1131 rheumatologists, respectively. 19 out of 20 SoCs were problematic from the perspectives of more than 20% of patients, while this was true for only 10 SoCs for rheumatologists. Rheumatologists in countries with lower gross domestic product and non-European Union countries were more likely to report problematic gaps in 15 of 20 SoCs, while virtually no differences were observed among patients. Lack of relevance of some SoCs (71%) and limited time of professionals (66%) were the most frequent implementation barriers identified by patients. CONCLUSIONS: Many problematic gaps were reported across several essential aspects of RA care. More efforts need to be devoted to implementation of EULAR SoCs.

Development of semiquantitative ultrasound scoring system to assess cartilage in rheumatoid arthritis.

OBJECTIVES: To develop and test the reliability of a new semiquantitative scoring system for the assessment of cartilage changes by ultrasound in a web-based exercise as well as a patient exercise of patients with RA. METHODS: A taskforce of the Outcome Measures in Rheumatology Ultrasound Working Group performed a systematic literature review on the US assessment of cartilage in RA, followed by a Delphi survey on cartilage changes and a new semiquantitative US scoring system, and finally a web-based exercise as well as a patient exercise. For the web-based exercise, taskforce members scored a dataset of anonymized static images of MCP joints in RA patients and healthy controls, which also contained duplicate images. Subsequently, 12 taskforce members used the same US to score cartilage in MCP and proximal interphalangeal joints of six patients with RA in in a patient reliability exercise. Percentage agreement and prevalence of lesions were calculated, as intrareader reliability was assessed by weighted kappa and interreader reliability by Light's kappa. RESULTS: The three-grade semiquantitative scoring system demonstrated excellent intrareader reliability (kappa: 0.87 and 0.83) in the web-based exercise and the patient exercise, respectively. Interreader reliability was good in the web-based exercise (kappa: 0.64) and moderate (kappa: 0.48) in the patient exercise. CONCLUSION: Our study demonstrates that ultrasound is a reliable tool for evaluating cartilage changes in the MCP joints of patients with RA and supports further development of a new reliable semiquantitative ultrasound scoring system for evaluating cartilage involvement in RA.

A reevaluation of Whittle (1986, 1992) reveals the link between detection thresholds, discrimination thresholds, and brightness perception.

In 1986, Paul Whittle investigated the ability to discriminate between the luminance of two small patches viewed upon a uniform background. In 1992, Paul Whittle asked subjects to manipulate the luminance of a number of patches on a uniform background until their brightness appeared to vary from black to white with even steps. The data from the discrimination experiment almost perfectly predicted the gradient of the function obtained in the brightness experiment, indicating that the two experimental methodologies were probing the same underlying mechanism. Whittle introduced a model that was able to capture the pattern of discrimination thresholds and, in turn, the brightness data; however, there were a number of features in the data set that the model couldn't capture. In this paper, we demonstrate that the models of Kane and Bertalmío (2017) and Kingdom and Moulden (1991) may be adapted to predict all the data but only by incorporating an accurate model of detection thresholds. Additionally, we show that a divisive gain model may also capture the data but only by considering polarity-dependent, nonlinear inputs following the underlying pattern of detection thresholds. In summary, we conclude that these models provide a simple link between detection thresholds, discrimination thresholds, and brightness perception.

Statistics of natural images as a function of dynamic range.

The statistics of real world images have been extensively investigated, but in virtually all cases using only low dynamic range image databases. The few studies that have considered high dynamic range (HDR) images have performed statistical analyses categorizing images as HDR according to their creation technique, and not to the actual dynamic range of the underlying scene. In this study we demonstrate, using a recent HDR dataset of natural images, that the statistics of the image as received at the camera sensor change dramatically with dynamic range, with particularly strong correlations with dynamic range being observed for the median, standard deviation, skewness, and kurtosis, while the one over frequency relationship for the power spectrum breaks down for images with a very high dynamic range, in practice making HDR images not scale invariant. Effects are also noted in the derivative statistics, the single pixel histograms, and the Haar wavelet analysis. However, we also show that after some basic early transforms occurring within the eye (light scatter, nonlinear photoreceptor response, center-surround modulation) the statistics of the resulting images become virtually independent from the dynamic range, which would allow them to be processed more efficiently by the human visual system.