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Objective: This study aimed to develop recommendations for communication and postgraduate education regarding primary care physical therapy for systemic sclerosis (SSc) patients. Methods: A virtual Nominal Group Technique was used with tasks forces for communication (n = 18) and education (n = 21). Both included rheumatologists, physical therapists (PTs) in primary, secondary or tertiary care, rheumatology nurses, advanced nurse practictioners and patient representatives. Three online meetings were organised for each task force to discuss (1) current bottlenecks; (2) potential solutions; and (3) the resulting draft recommendations. After the final adjustments, participants rated their level of agreement with each recommendation on a scale from 0 (not at all agree) to 100 (totally agree), using an online questionnaire. Results: 19 and 34 recommendations were formulated for communication and education, respectively. For communication the main recommendations concerned the provision of an overview of primary care physical therapists with expertise in rheumatic and musculoskeletal diseases to patients and rheumatologists, the inclusion of the indication by the rheumatologist in the referral to the physical therapist and low‐threshold communication with the rheumatologist in case of questions or concerns of the physical therapist. For postgraduate education three types of "on demand" educational offerings were recommended with varying levels of content and duration, to match the competencies and preferences of individual primary care physical therapists. Conclusion: Using a systematic qualitative approach, two multi‐stakeholder task forces developed practical recommendations for primary care physical therapists' communication with hospital‐based care providers and postgraduate education regarding the treatment of SSc patients. [ABSTRACT FROM AUTHOR]

Objectives Silver fibre gloves transport heat from the palm to the fingers, possibly reducing the burden of RP in SSc patients. We aim to evaluate the clinical efficiency of this intervention. Methods A multicentre, double-blind, randomized trial was performed, accounting for interindividual differences and external factors using a crossover design. Patients were randomized in two groups: group 1 wore 8% silver fibre gloves in period 1 and normal gloves in period 2 and group 2 vice versa. Each period lasted 6 weeks. The primary outcome was the Raynaud Condition Score (RCS) over time (minimal clinical important difference 1.4), assessed three times per week using an online questionnaire. Secondary outcomes included vascular complications and Scleroderma-Health Assessment Questionnaire (SHAQ). Outcomes were evaluated before unblinding using linear mixed models. Results A total of 85 SSc patients were included, with 76 completing the study. The mean RCS during 2 weeks before the study (i.e. without gloves) was 6.4 (s. d. 1.6). Both with silver fibre gloves and normal gloves the mean RCS decreased to 3.9 (s. d. 2.3) with a similar course over time. There was no difference in mean RCS over time between the type of gloves [β = 0.067 (95% CI −0.006, 0.19)]. Of secondary outcomes, total SHAQ [β = 0.036 (95% CI 0.026, 0.046)] was slightly higher with silver fibre gloves, which is clinically irrelevant. Three patients developed new digital ulcers with normal gloves vs one patient with silver fibre gloves [odds ratio 3.2 (95% CI 0.32, 31.1)]. Conclusions Wearing gloves in SSc patients clearly decreases the RP burden. Our results do not support the hypothesis that increased heat transport of 8% silver fibre gloves is associated with less disease burden as measured in this study by the RCS compared with normal gloves. Clinical trial registration number Netherlands Trial register (https://www.trialregister.nl/) NL7904 [ABSTRACT FROM AUTHOR]

Objective This study aimed to determine whether lower values of feature-tracking cardiovascular magnetic resonance (CMR)-derived left atrial reservoir strain (LARS) and impaired left ventricular (LV) global longitudinal strain (GLS) were associated with the presence of symptoms and long-term prognosis in patients with SSc. Methods A total of 100 patients {54 [interquartile range (IQR) 46–64] years, 42% male} with SSc who underwent CMR imaging at two tertiary referral centres were included. All patients underwent analysis of LARS and LV GLS using feature-tracking on CMR and were followed-up for the occurrence of all-cause mortality. Results The median LV GLS was –21.8% and the median LARS was 36%. On multivariable logistic regression, LARS [odds ratio (OR) 0.964 per %, 95% CI 0.929, 0.998, P  = 0.049] was independently associated with New York Heart Association (NYHA) class II–IV heart failure symptoms. Over a median follow-up of 37 (21–62) months, a total of 24 (24%) patients died. Univariable Cox regression analysis demonstrated that LARS [hazard ratio (HR) 0.94 per 1%, 95% CI 0.91, 0.97, P  < 0.0001) and LV GLS (HR 1.10 per %, 95% CI 1.03, 1.17, P  = 0.005) were associated with all-cause mortality, while LV ejection fraction was not. Likelihood ratio tests demonstrated that LARS provided incremental value over prognostically important clinical and imaging parameters, including late gadolinium enhancement. Conclusion In patients with SSc, LARS was independently associated with the presence of NYHA class II–IV heart failure symptoms. Although both LARS and LV GLS were associated with all-cause mortality, only LARS provided incremental value over all evaluated variables known to be prognostically important in patients with SSc. [ABSTRACT FROM AUTHOR]

Objectives In SSc patients, disease specific determinants that influence health-related quality of life (HRQoL) over time have not been described. We aim to, in patients with SSc, (i) evaluate if and how HRQoL changes over time, and (ii) assess how different SSc domains and functional impairments contribute to changes in HRQoL over time. Methods All SSc patients from the Leiden SSc cohort were included; patients with disease duration <24 months were classified as incident cases. HRQoL was assessed prospectively on an annual basis using the EQ-5D and the SF36. To assess baseline associations between clinical characteristics and HRQoL, linear regressions were performed. To identify possible associations between SSc characteristics and HRQoL change over time, linear mixed models were performed in both incident and prevalent cases. Results In total, 492 SSc patients were included (n  = 202 incident cases), with a median follow-up duration of 3.4 years. At baseline, presence of organ involvement was independently associated with a worse SF36 physical component score and lower EQ-5D score. Over time, gastrointestinal symptoms, Raynaud and digital ulcers were independently associated with deterioration of HRQoL in both incident and prevalent cases. In prevalent cases, pulmonary arterial hypertension (PAH) was associated with a decrease in HRQoL over time. Worse functioning as measured by six-min walking distance, mouth-opening, finger-to-palm distance and grip-strength contributed significantly to deterioration of HRQoL over time. Conclusion In SSc, key clinical burdens that contribute to worsening of HRQoL over time include digital ulcers, Raynaud and gastrointestinal involvement. In addition, PAH is a significant burden in prevalent disease. [ABSTRACT FROM AUTHOR]

In the article, the authors present their study which examined the disease progression in systemic sclerosis (SSc), a heterogeneous disease wherein vasculopathy and fibrosis affect various organs like heart, lungs, and kidneys. The study used the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) criteria in SSc to assess its cardiac progression, lung progression, and skin progression.

Metacognitive skills regulate and control learning processes. A developmental study (Van der Stel & Veenman, 2014) revealed that metacognitive growth is interrupted at the age of 14-15 years, while metacognitive skills are generalized over tasks and domains at the same time. The present study seeks to confirm this pause or decline in metacognitive growth, however, with a gender-age interaction. Females are expected to run one year ahead of males in metacognitive development. Additionally, the usefulness of computer-logfile analysis as an unobtrusive method for assessing metacognitive development is investigated. A hundred and nineteen secondary-school students (66 male; 53 female) at the age of 13 to 16 years performed a computerized inductive-learning task. Traces of learner activities were stored in logfiles and automatically scored on metacognitive skills. Afterwards, participants completed a learning posttest. Results substantiate the expected gender-age interaction in the metacognition data. Females started low at 14 years, recovered at 15 years, and peaked at 16 years, whereas males started positive at 14 years, declined at 15 years, and recovered at 16 years. Posttest data show a significant effect of age with improved learning performance at 16 years. Implications for the study of metacognitive development are discussed. [ABSTRACT FROM AUTHOR]

Aims: Epicardial adipose tissue (EAT) has emerged as a mediator between systemic inflammatory disorders and cardiovascular disease, and may therefore play a role in the pathophysiology of cardiac involvement in systemic sclerosis (SSc). The aim of this study was to assess the correlation between EAT and left ventricular (LV) function, and to determine the prognostic value of EAT in patients with SSc., Methods and Results: Consecutive patients with SSc who underwent non-contrast thorax computed tomography and echocardiography were included. EAT mass was quantified using dedicated software. The study endpoint was all-cause mortality. A total of 230 SSc patients (age 53 ± 15 years, 14% male) were included. The median value of EAT mass was 67 g (interquartile range: 45-101 g). Patients with increased EAT mass (≥67 g) showed more impaired LV diastolic function as compared with patients with less EAT mass (<67 g), and even after adjusting for age and comorbidities, EAT mass was independently associated with LV diastolic function parameters. During a median follow-up of 8 years, 42 deaths occurred. Kaplan-Meier analysis showed that patients with increased EAT mass had higher all-cause mortality rate as compared with patients with less EAT mass (29% vs. 7%; P < 0.001). In the multivariable analysis, EAT was independently associated with all-cause mortality after adjusting for important covariates (HR: 1.006; 95% CI: 1.001-1.010)., Conclusion: In patients with SSc, EAT is independently associated with LV diastolic dysfunction and higher mortality rate., Competing Interests: Conflict of interest: The Department of Cardiology of the Leiden University Medical Center received unrestricted research grants from Abbott Vascular, Alnylam, Bayer, Bioventrix, Biotronik, Boston Scientific, Edwards Lifesciences, GE Healthcare, and Medtronic. V.D. received speaker fees from Abbott Vascular, Edwards Lifesciences, GE Healthcare, MSD, Novartis, and Medtronic. J.J.B. received speaker fees from Abbot Vascular and Edwards Lifesciences. N.A.M. received speaker fees from GE Healthcare, Philips Ultrasound, and Abbott Vascular, and research grant from Alnylam. J.K.D.V.-B. received consulting fees from Abbvie, Janssen, and Boehringer Ingelheim, and received research grants from ReumaNederland, NVLE, Roche, Galapagos, and Janssen. All payments were made to her institution. The remaining authors have nothing to disclose., (© The Author(s) 2023. Published by Oxford University Press on behalf of the European Society of Cardiology.)

A hallmark of disease pathogenesis of systemic sclerosis (SSc) is the presence of autoreactive B cell responses targeting nuclear proteins. Almost all SSc-patients harbour circulating antinuclear autoantibodies of which anti-topoisomerase 1, anti-centromere protein, anti-RNA polymerase III and anti-fibrillarin autoantibodies (ATA, ACA, ARA and AFA, respectively) are the most common and specific for SSc. In clinical practice, autoantibodies serve as diagnostic biomarkers and can aid in the identification of clinical phenotypes of the disease. However, factors driving disease progression in SSc are still poorly understood, and it is difficult to predict disease trajectories in individual patients. Moreover, treatment decisions remain rather empirical, with variable response rates in clinical trials due to patient heterogeneity. Current evidence has indicated that certain patients may benefit from B cell targeting therapies. Hence, it is important to understand the contribution of the antinuclear autoantibodies and their underlying B cell response to the disease pathogenesis of SSc., (Copyright © 2022 The Author(s). Published by Elsevier Inc. All rights reserved.)

Objectives: Haematopoietic stem cell transplantation (HSCT) is a treatment option for patients with severe systemic sclerosis (SSc), but the efficacy of the procedure in remodelling the nailfold microvascular array is largely unknown. Therefore, this study aimed to evaluate the effect of HSCT on microangiopathy assessed through nailfold capillaroscopy (NC) and to compare the results with findings in patients receiving conventional immunosuppression., Methods: We included SSc patients with severe SSc and whose pre- and post-treatment NC images were available. Findings in patients treated with HSCT were compared with patients not treated with HSCT. Images were scored by two independent observers blinded for clinical data and treatment history. Capillary pattern was determined and semiquantitative scores from 0 (no changes) to 3 (>66% alterations per millimetre) were used to quantify the degree of specific microvascular characteristics. Changes in severity of microangiopathy between baseline and post-treatment were compared between groups., Results: Images of 18 HSCT patients and 21 controls were scored. From baseline to follow-up, 33% of HSCT patients showed improvement from scleroderma pattern to normal NC, compared to 6% of controls (p=0.15). Pre- to post-treatment differences in semiquantitative scores showed significant improvement in HSCT patients compared to controls regarding capillary loss (-0.5 vs. 0.0, p<0.05) and disorganisation (-0.8 vs. 0.0, p<0.05)., Conclusions: The degree of microangiopathy improved significantly in severe SSc patients treated with HSCT compared with patients receiving conventional immunosuppressive therapy.

Objectives: Health-Related Quality of Life (HRQoL) in adult patients with mixed connective tissue disease (MCTD) has not been described so far. Therefore, we performed an explorative study to evaluate HRQoL in MCTD patients., Methods: MCTD patients fulfilling the Kahn criteria and participating in the prospective follow-up cohort for MCTD of the Leiden University Medical Center were included; and matched to systemic sclerosis (SSc) patients based on age, sex and disease duration. Data on disease characteristics and HRQoL (SF36 and EQ-5D) were collected annually. HRQoL was compared between MCTD and SSc patients at baseline. Factors associated with HRQoL in MCTD were identified using linear regression and change in HRQoL over 3 years using linear mixed models., Results: Thirty-four MCTD patients (121 visits) and 102 SSc patients (424 visits) were included. At baseline, MCTD patients presented with interstitial lung disease, cardiac involvement, synovitis and myositis more frequently compared to SSc patients, while use of immunosuppressive medication was less frequent. In both groups, mean SF36 scores were lower than in the general Dutch population. The SF36 subscore "general health perception" was impacted most in both groups (MCTD: 38.5 [SD:7.0], SSc: 39.9 [SD:8.9]). During follow-up, SF36 scores improved in MCTD patients, while EQ5DNL remained stable. No specific characteristics were identified that associated with baseline HRQoL or change in HRQol over time., Conclusions: Like in SSc, HRQoL in MCTD is significantly impaired, especially the general health perception of patients. Evaluation in larger prospective cohorts is needed to identify characteristics that impact HRQol most.

In systemic sclerosis (SSc) therapeutic efforts are often directed to prevent progressive respiratory impairment, but it is unclear to what extent changes in pulmonary function tests (PFTs) are associated with health-related quality of life (HRQoL). The aim of our study is to evaluate how modifications in PFTs contribute to longitudinal variations in HRQoL, assessed through the multidimensional questionnaire EQ-5D, in patients with SSc. We included SSc patients with forced vital capacity (FVC%), diffusing capacity of the lungs for carbon monoxide (DLCO%) and EQ-5D assessed in at least two visits. The EQ-5D consists of two parts, a utility score ranging from - 0.59 to 1, and a 0-100 Visual Analogue Scale (VAS). Higher values represent better health. The association between changes in FVC% and DLCO%, and evolution of EQ-5D over time, was investigated using generalized estimating equations. Three hundred seventy-eight patients were included, accounting for a total of 1619 measurements. The models showed that improvement in FVC% is significantly associated with increase in both utility score (β = 0.001; 95% CI 0.000 to 0.002; p = 0.003) and VAS over time (β = 0.188; 95% CI 0.111 to 0.264; p < 0.001). Moreover, improvement in DLCO% is longitudinally associated with increase in utility score (β = 0.001; 95% CI 0.000 to 0.002; p = 0.038), while the results for VAS were non-significant (β = 0.020; 95% CI -0.079 to 0.120; p = 0.690). We show that change in PFTs has a significant, although minor, impact on HRQoL as measured by EQ-5D in SSc.Key Points• In patients with SSc, changes in PFTs have a significant, although minor, impact on HRQoL.• In patients with SSc-ILD, the perception of HRQoL is nearly not influenced by changes in pulmonary function.• The use of generic questionnaires might not be sensitive enough to evaluate the impact on quality of life of therapies targeting specific SSc manifestations.