[Methods for assessment of disease activity of polymyalgia rheumatica]. / Methoden zur Erfassung der Krankheitsaktivität der Polymyalgia rheumatica.

Polymyalgia rheumatica (PMR) is the second most frequent inflammatory rheumatic disease in old age. Remission and recurrence are frequently used as endpoints in clinical trials; however, there is as yet no international consensus on the definition of these states, which limits the comparability of published studies. The PMR activity score (PMR-AS) is the only composite score specifically developed for PMR, which together with remission is used to define low, middle and high disease activity. In recent studies the PMR-AS was often used and low disease activity was established as endpoint. The most important limitation of the PMR-AS is the potential influence of the individual variables by comorbidities. The value of C­reactive protein (CRP) and the erythrocyte sedimentation rate (ESR) are of restricted value in studies using drugs that influence the interleukin 6 (IL-6) axis. In these cases, calprotectin and osteopontin are promising alternative biomarkers, as they have already been shown to reflect disease activity independently of CRP in rheumatoid arthritis. Furthermore, imaging modalities including sonography, magnetic resonance imaging and fluorodeoxyglucose (FDG) positron emission tomography could also be helpful in monitoring disease activity; however, these techniques must first be validated in further studies. The PMR impact scale (PMR-IS) is a composite score to assess the impact of PMR on the patients; however, it has not yet been used in clinical studies. The development of additional patient reported outcomes (PRO) for PMR and the definition of standardized criteria for documentation of remission and recurrence are important questions in the future research agenda for PMR.

2022 EULAR points to consider for remote care in rheumatic and musculoskeletal diseases.

BACKGROUND: Remote care and telehealth have the potential to expand healthcare access, and the COVID-19 pandemic has called for alternative solutions to conventional face-to-face follow-up and monitoring. However, guidance is needed on the integration of telehealth into clinical care of people with rheumatic and musculoskeletal diseases (RMD). OBJECTIVE: To develop EULAR points to consider (PtC) for the development, prioritisation and implementation of telehealth for people with RMD. METHODS: A multidisciplinary EULAR task force (TF) of 30 members from 14 European countries was established, and the EULAR standardised operating procedures for development of PtC were followed. A systematic literature review was conducted to support the TF in formulating the PtC. The level of agreement among the TF was established by anonymous online voting. RESULTS: Four overarching principles and nine PtC were formulated. The use of telehealth should be tailored to patient's needs and preferences. The healthcare team should have adequate equipment and training and have telecommunication skills. Telehealth can be used in screening for RMD as preassessment in the referral process, for disease monitoring and regulation of medication dosages and in some non-pharmacological interventions. People with RMD should be offered training in using telehealth, and barriers should be resolved whenever possible.The level of agreement to each statement ranged from 8.5 to 9.8/10. CONCLUSION: The PtC have identified areas where telehealth could improve quality of care and increase healthcare access. Knowing about drivers and barriers of telehealth is a prerequisite to successfully establish remote care approaches in rheumatologic clinical practice.

The role of tender and swollen joints for the assessment of inflammation in PsA using ultrasound.

OBJECTIVE: To evaluate tender joints (TJ) and swollen joints (SJ) for the assessment of ultrasound (US) defined inflammation in PsA. METHODS: Eighty-three PsA patients underwent clinical and US examinations at two scheduled study visits 12 months apart. Tenderness and swelling were assessed at 68 and 66 joints, respectively, and US examinations were conducted at all 68 joints. At patient level, associations with clinical composites and US scores were performed using correlations and by analysing patients with predominantly tender (pTender) or swollen joints (pSwollen). At joint level, a Power Doppler (PD) value ≥ 1 was defined as active synovitis. A generalized linear mixed model was created to assess the predictive value of TJ and SJ for active synovitis after 12 months. RESULTS: SJC showed better correlations with GS/PD scores (r = 0.37/0.47) than with TJC (PD: r = 0.33), while TJC correlated better with patient reported outcomes (PROMs) like patient global assessment (TJC: r = 0.57; SJC r = 0.39). Patients with pTender showed poorer results for PROMs and disease activity scores than patients with pSwollen, but not for laboratory or US markers of inflammation. Swollen joints showed active synovitis in 35% of cases, while only 16% of tender joints were active according to US. Swelling at baseline better predicted active synovitis at the same joint after 12 months [odds ratio (OR) 6.33, P <0.001] as compared with tenderness (OR 3.58, P <0.001). CONCLUSIONS: SJ are more closely linked with US signs of inflammation as compared with TJ in PsA. Joint swelling is a better predictor for signs of US inflammation than tenderness after one year of follow-up.

OMERACT definition and reliability assessment of chronic ultrasound lesions of the axillary artery in giant cell arteritis.

OBJECTIVES: To define chronic ultrasound lesions of the axillary artery (AA) in long-standing giant cell arteritis (GCA) and to evaluate the reliability of the new ultrasound definition in a web-based exercise. METHODS: A structured Delphi, involving an expert panel of the Large Vessel Vasculitis subgroup of the Outcome Measures in Rheumatology (OMERACT) Ultrasound Working Group was carried out. The reliability of the new definition was tested in a 2-round web-based exercise involving 23 experts and using 50 still images each from AA of long-standing and acute GCA patients, as well as 50 images from healthy individuals. RESULTS: The final OMERACT ultrasound definition of chronic changes was based on measurement and appearance of the intima-media complex. The overall reliability of the new definition for chronic ultrasound changes in longstanding GCA of the AA was good to excellent with Light's kappa values of 0.79-0.80 for inter-reader reliability and mean Light's-kappa of 0.88 for intra-reader reliability. The mean inter-rater and intra-rater agreements were 86-87% and 92%, respectively. Good reliabilities were observed comparing the vessels with longstanding versus acute GCA with a mean agreement and kappa values of 81% and 0.63, respectively. CONCLUSION: The new OMERACT ultrasound definition for chronic vasculitis of the AA in GCA revealed a good to excellent inter- and intra-reader reliability in a web-based exercise of experts.

Data linkage analysis of giant cell arteritis in Italy: Healthcare burden and cost of illness in the Italian region of Friuli Venezia Giulia (2001-2017).

Giant cell arteritis (GCA) is the most common vasculitis in adults. However, comprehensive analyses of the healthcare burden are still scarce. The aim of the study is to report the healthcare burden and cost of illness of GCA in the Friuli Venezia Giulia (FVG) region of Italy, based on a data linkage analysis. To this end, a retrospective study was conducted through the integration of many administrative health databases of the FVG region as the source of information. Cases were identified from two verified, partially overlapping sources (the rare disease registry and medical exemption database). From 2001 to 2017, 208 patients with GCA were registered. The prevalence of GCA in the population aged ⩾ 45 years as of December 31, 2017 was 27.2/100,000 inhabitants (95% CI 23.5-31.4). The mean time of observation was 4.5 ± 3.6 years. A total of 3182 visits (338 per 100 patient-years) was recorded. The most frequent specialty visits were rheumatology (n = 610, 19.2%), followed by internal medicine (n = 564, 17.7%). A total of 287 hospitalizations (30 per 100 patient-years) were reported. A total of 13,043 prescriptions (1386 per 100 patient-years) were registered. More than half of the patients were prescribed an immunosuppressive agent. The overall estimated direct healthcare cost was €2,234,070, corresponding to €2374 per patient-year. Overall, GCA is a rare disease which implies a high healthcare cost.

Systematic literature review informing the 2018 update of the EULAR recommendation for the management of large vessel vasculitis: focus on giant cell arteritis.

Objectives: To analyse the current evidence for the management of large vessel vasculitis (LVV) to inform the 2018 update of the EULAR recommendations. Methods: Two systematic literature reviews (SLRs) dealing with diagnosis/monitoring and treatment strategies for LVV, respectively, were performed. Medline, Embase and Cochrane databases were searched from inception to 31 December 2017. Evidence on imaging was excluded as recently published in dedicated EULAR recommendations. This paper focuses on the data relevant to giant cell arteritis (GCA). Results: We identified 287 eligible articles (122 studies focused on diagnosis/monitoring, 165 on treatment). The implementation of a fast-track approach to diagnosis significantly lowers the risk of permanent visual loss compared with historical cohorts (level of evidence, LoE 2b). Reliable diagnostic or prognostic biomarkers for GCA are still not available (LoE 3b).The SLR confirms the efficacy of prompt initiation of glucocorticoids (GC). There is no high-quality evidence on the most appropriate starting dose, route of administration, tapering and duration of GC (LoE 4). Patients with GCA are at increased risk of dose-dependent GC-related adverse events (LoE 3b). The addition of methotrexate or tocilizumab reduces relapse rates and GC requirements (LoE 1b). There is no consistent evidence that initiating antiplatelet agents at diagnosis would prevent future ischaemic events (LoE 2a). There is little evidence to guide monitoring of patients with GCA. Conclusions: Results from two SLRs identified novel evidence on the management of GCA to guide the 2018 update of the EULAR recommendations on the management of LVV.

Video clip assessment of a salivary gland ultrasound scoring system in Sjögren's syndrome using consensual definitions: an OMERACT ultrasound working group reliability exercise.

OBJECTIVE: To develop ultrasound (US) definitions and a US novel scoring system for major salivary gland (SG) lesions in patients with primary Sjögren's syndrome (pSS) and to test their intrareader and inter-reader reliability using US video clips. METHODS: Twenty-five rheumatologists were subjected to a three-round, web-based Delphi process in order to agree on (1) definitions and scanning procedure of salivary gland ultrasonography (SGUS): parotid, submandibular and sublingual glands (PG, SMG and SLG); (2) definitions for the elementary SGUS lesions in patients with Sjögren's syndrome; (3) scoring system for grading changes. The experts rated the statements on a 1-5 Likert scale. In the second step, SGUS video clips of patients with pSS and non-pSS sicca cases were collected containing various spectrums of disease severity followed by an intrareader and inter-reader reliability exercise. Each video clip was evaluated according to the agreed definitions. RESULTS: Consensual definitions were developed after three Delphi rounds. Among the three selected SGs, US assessment of PGs and SMGs was agreed on. Agreement was reached to score only greyscale lesions and to focus on anechoic/hypoechoic foci in a semiquantitative matter or, if not possible on a qualitatively (present/absent) evaluation of fatty or fibrous lesions. Intrareader reliability for detecting and scoring these lesions was excellent (Cohen's kappa 0.81) and inter-reader reliability was good (Light's kappa 0.66). CONCLUSION: New definitions for developing a novel semiquantitative US score in patients with pSS were developed and tested on video clips. Inter-reader and intrareader reliabilities were good and excellent, respectively.

Definitions and reliability assessment of elementary ultrasound lesions in giant cell arteritis: a study from the OMERACT Large Vessel Vasculitis Ultrasound Working Group.

OBJECTIVES: To define the elementary ultrasound (US) lesions in giant cell arteritis (GCA) and to evaluate the reliability of the assessment of US lesions according to these definitions in a web-based reliability exercise. METHODS: Potential definitions of normal and abnormal US findings of temporal and extracranial large arteries were retrieved by a systematic literature review. As a subsequent step, a structured Delphi exercise was conducted involving an expert panel of the Outcome Measures in Rheumatology (OMERACT) US Large Vessel Vasculitis Group to agree definitions of normal US appearance and key elementary US lesions of vasculitis of temporal and extracranial large arteries. The reliability of these definitions on normal and abnormal blood vessels was tested on 150 still images and videos in a web-based reliability exercise. RESULTS: Twenty-four experts participated in both Delphi rounds. From originally 25 statements, nine definitions were obtained for normal appearance, vasculitis and arteriosclerosis of cranial and extracranial vessels. The 'halo' and 'compression' signs were the key US lesions in GCA. The reliability of the definitions for normal temporal and axillary arteries, the 'halo' sign and the 'compression' sign was excellent with inter-rater agreements of 91-99% and mean kappa values of 0.83-0.98 for both inter-rater and intra-rater reliabilities of all 25 experts. CONCLUSIONS: The 'halo' and the 'compression' signs are regarded as the most important US abnormalities for GCA. The inter-rater and intra-rater agreement of the new OMERACT definitions for US lesions in GCA was excellent.

Value of Multiparametric US in the Assessment of Intratesticular Lesions.

Purpose To evaluate the diagnostic accuracy of multiparametric ultrasonography (US) consisting of gray-scale US, color Doppler US, strain elastography, and contrast agent-enhanced US in the assessment of intratesticular lesions. Materials and Methods Institutional review board approval was obtained for this retrospective study. From January 2012 to December 2015, 55 focal testicular lesions that were indeterminate on gray-scale US scans were further characterized with color Doppler US, strain elastography, and contrast-enhanced US. Strain elastography was performed to assess tissue elasticity, and hard lesions were defined as malignant. Color Doppler US and contrast-enhanced US were performed to determine the absence or presence of vascularization. Avascular lesions were defined as benign. Histopathologic results or follow-up examinations served as reference standards. Correct classification rate, sensitivity, specificity, and likelihood ratio were calculated. Results Of 55 testicular lesions, 43 (78.2%) were benign and 12 (21.8%) were malignant. Single-modality sensitivities and specificities were 66.7% and 88.4% for color Doppler US, 100% and 76.7% for contrast-enhanced US, and 100% and 72.1% for strain elastography, respectively. Among 12 malignant lesions, color Doppler US failed to demonstrate vascularization in four (33.3%) lesions, which were positive for cancer at contrast-enhanced US. By combining strain elastography and contrast-enhanced US, a sensitivity of 100% and specificity of 93.0% were achieved in differentiating benign and malignant focal testicular lesions. Positive likelihood ratio was 5.7 for color Doppler US, 4.3 for contrast-enhanced US, 3.6 for strain elastography, 14.3 for strain elastography combined with color Doppler US, and 14.3 for strain elastography combined with contrast-enhanced US. Conclusion Multiparametric US allows for a reliable differentiation of benign and malignant intratesticular lesions and can potentially be useful in deciding whether orchiectomy can be replaced with follow-up or less invasive organ-sparing strategies. © RSNA, 2017.

Rheumatology Workforce Planning in Western Countries: A Systematic Literature Review.

OBJECTIVE: To compare health care planning models forecasting rheumatology workforce requirements in western countries. METHODS: A systematic literature review was conducted through medical databases (Ovid MEDLINE, Embase, CINAHL, and Cochrane Library) and the grey literature. All articles reporting a rheumatology workforce model were included. RESULTS: The search yielded 6,508 articles, and 14 publications (on 12 studies) were included. Workforce models were available for the US (n = 3), Canada (n = 3), the US plus Canada (n = 1), Germany (n = 2), Spain (n = 1), and the UK (n = 2). The number of rheumatologists required to serve a population of 100,000 people was calculated, with a range of 0.7 (UK, calculated for 1988) to 3.5 (Spain, calculated for 2021). Most models used a needs-based approach (n = 6); 3 studies each applied a supply- or demand-based method. The following variables were considered by ≥1 model: disease prevalence, patients' referral to rheumatologists, clinical visits/patient/year, population development, factors influencing performance of rheumatologists, patient flow/care sharing, and medical technologies/infrastructure development. CONCLUSION: Heterogeneity in methods used, the period or calendar years for which the estimates were projected, and heterogeneity of variables evaluated led to disparate estimates, with results ranging from 0.7 to 3.5 rheumatologists per 100,000 population. An international initiative is needed to agree upon a common approach for a reliable estimation of manpower requirements in rheumatology.

Ultrasound composite scores for the assessment of inflammatory and structural pathologies in Psoriatic Arthritis (PsASon-Score).

INTRODUCTION: This study was performed to develop ultrasound composite scores for the assessment of inflammatory and structural lesions in Psoriatic Arthritis (PsA). METHODS: We performed a prospective study on 83 PsA patients undergoing two study visits scheduled 6 months apart. B-mode and Power Doppler (PD) findings were semi-quantitatively scored at 68 joints (evaluating synovia, perisynovial tissue, tendons and bone) and 14 entheses. We constructed bilateral and unilateral (focusing the dominant site) ultrasound composite scores selecting relevant sites by a hierarchical approach. We tested convergent construct validity, reliability and feasibility of inflammatory and structural elements of the scores as well as sensitivity to change for inflammatory items. RESULTS: The bilateral score (termed PsASon22) included 22 joints (6 metacarpophalangeal joints (MCPs), 4 proximal interphalangeal joints (PIPs) of hands (H-PIPs), 2 metatarsophalangeal joints (MTPs), 4 distal interphalangeal joints (DIPs) of hands (H-DIPs), 2 DIPs of feet (F-DIPs), 4 large joints) and 4 entheses (bilateral assessment of lateral epicondyle and distal patellar tendon). The unilateral score (PsASon13) compromised 13 joints (2 MCPs, 3 H-PIPs, 1 PIP of feet (F-PIP), 2 MTPs, 1 H-DIP and 2 F-DIPs and 2 large joints) and 2 entheses (unilateral lateral epicondyle and distal patellar tendon). Both composite scores revealed a moderate to high sensitivity (bilateral composite score 43% to 100%, unilateral 36% to 100%) to detect inflammatory and structural lesions compared to the 68-joint/14-entheses score. The inflammatory and structural components of the composite scores correlated weakly with clinical markers of disease activity (corrcoeffs 0 to 0.40) and the health assessment questionnaire (HAQ, corrcoeffs 0 to 0.39), respectively. Patients with active disease achieving remission at follow-up yielded greater reductions of ultrasound inflammatory scores than those with stable clinical activity (Cohen's d effect size ranging from 0 to 0.79). Inter-rater reliability of bi- and unilateral composite scores was moderate to good with ICCs ranging from 0.42 to 0.96 and from 0.36 to 0.71, respectively for inflammatory and structural sub-scores. The PsASon22 and PsASon13 required 16 to 26 and 9 to 13 minutes, respectively to be completed. CONCLUSION: Both new PsA ultrasound composite scores (PsASon22 and PsASon13) revealed sufficient convergent construct validity, sensitivity to change, reliability and feasibility.

Real-time sonoelastography of salivary glands for diagnosis and functional assessment of primary Sjögren's syndrome.

The purpose of this study was to investigate the value of real-time sonoelastography (RTS) of salivary glands for the diagnosis and assessment of glandular damage in primary Sjögren's syndrome (pSS). After institutional review board approval, 45 pSS patients, 24 sicca patients and 11 healthy controls were investigated prospectively. Questionnaires were completed and Saxon and Schirmer tests and routine blood tests carried out in all patients. All patients underwent B-mode ultrasonography and RTS of parotid and submandibular glands. Abnormal findings were graded from 0 to 48 and from 0 to 16, respectively. Sialoscintigraphy was done according to a routine protocol; scoring ranged from 0 to 12. Statistical analysis comprised receiver operating characteristic curve and multivariate regression analysis. Patients with pSS had higher B-mode (median score = 25 [range: 2-44] vs. 9 [1-20], p < 0.001) and RTS (6.5 [2-13] versus 4 [1-9], p < 0.001) scores than controls with sicca syndrome, yielding areas under the curve of 0.83 and 0.85 (p < 0.05 each), respectively for pSS diagnosis. In cases with an inconclusive B-mode ultrasonography result, RTS (cutoff score: ≥ 6) led to a sensitive (66.7%) and specific (85.7%) classification of patients and sicca controls. In multivariate regression analysis, RTS (regression coefficient = -0.48, p = 0.005), but not B-mode ultrasonography, reflected impaired salivary gland function according to the Saxon test, whereas none of the subjective measures of dryness or discomfort were related to ultrasonography results. B-mode and RTS results were both associated with sialoscintigraphy scores (regression coefficient = 0.66, p < 0.001, and regression coefficient = 0.55, p = 0.001, respectively). Reproducibility of B-mode ultrasonography and RTS was good, with intra-class correlation coefficients of 0.93 (95% confidence interval: 0.57-0.98) and 0.93 (95% confidence interval: 0.79-0.98), respectively. In summary, RTS might be a useful adjunct to B-mode ultrasonography for diagnosis and assessment of salivary gland impairment in primary Sjögren's syndrome.

Clinical implementation of musculoskeletal ultrasound in rheumatology in Austria.

The aim of the study is to assess the clinical implementation of musculoskeletal ultrasound (MSUS) in rheumatology in Austria. A survey was conducted among Austrian rheumatologists and physicians of other specialties with a focus on rheumatology. The questionnaire was designed by the members of the Austrian Radiology-Rheumatology Initiative for Musculoskeletal UltraSound including the following items: demographics, access to MSUS and MSUS training, application of MSUS to support diagnosis, monitoring and treatment decisions, and obstacles for the routine performance of MSUS. Eighty-eight (21.9 %) out of the 402 surveyed physicians responded. No access to MSUS and/or inadequate training in the technique was more commonly reported by senior (>50 years; 64.3 and 67.7 %, respectively) than by younger physicians (16.7 %, p = 0.01 and 18.5 %, p < 0.001, respectively). The lowest availability of sonography was found among senior rheumatologists (25.0 %, p = 0.001 compared to the total group). MSUS is routinely used for diagnosis and/or monitoring purposes by 12.5 % of physicians and 20.5 % perform sonography in clinically unclear cases. A limited number of physicians apply the method to support treatment decisions and/or to evaluate treatment success. The most important obstacles for routine application of MSUS in rheumatology are limited access to ultrasound machines, lack of training/education in the technique, and time constraints in daily routine. Low access to high-end ultrasound devices, lack of training, and time constraints may explain the low appreciation of MSUS among Austrian physicians evaluating patients with rheumatic diseases.