Evaluation of the European Society of Cardiology Risk Assessment Score in Incident Systemic Sclerosis-Associated Pulmonary Arterial Hypertension.

OBJECTIVE: Patients with pulmonary arterial hypertension (PAH) may be stratified as low, intermediate, or high risk of 1-year mortality. In 2022, the European Society of Cardiology (ESC) updated and simplified its risk stratification tool, based on three variables: World Health Organization functional class, serum N-terminal pro-brain type natriuretic peptide and six-minute walk distance, applied at follow-up visits, intended to guide therapy over time. METHODS: We applied the 2022 ESC risk assessment tool at baseline and follow-up (within 2 years) to a multinational incident cohort of systemic sclerosis-associated PAH (SSc-PAH). Kaplan-Meier curves, Cox hazards regression, and accelerated failure time models were used to evaluate survival by risk score. RESULTS: At baseline (n = 260), the majority of SSc-PAH (72.2%) were graded as intermediate risk of death according to the 2022 tool. At follow-up, according to 2022 tool, half (55.5%) of the cohort were classified as low or intermediate-low risk. The 2022 risk model at follow-up was able to differentiate survival between risk strata. All three individual parameters (World Health Organization functional class, N-terminal pro-brain type natriuretic peptide, six-minute walk distance) were significantly associated with mortality at baseline and/or follow-up. CONCLUSION: The 2022 ESC risk assessment strategy applied at baseline and follow-up predicts survival in SSc-PAH. Treatment decisions for SSc-PAH should include risk assessments, aiming to achieve low-risk status according to the 2022 ESC guidelines.

Social cognitive theory to improve symptom appraisal and help-seeking among patients with autoimmune rheumatic diseases: A qualitative study.

AIM: Social cognitive theory (SCT) has been successfully employed to improve symptom appraisal and help-seeking among patients with various conditions but is yet to be applied in the context of autoimmune rheumatic diseases (ARDs). This study aimed to explore the applicability of SCT in and possible approaches to improving symptom appraisal and help-seeking of patients with ARDs, one of the key barriers to earlier diagnosis. METHODS: Semi-structured interviews were conducted with 33 ARD patients with a prolonged pre-diagnosis interval (>3 months). We coded the transcripts deductively using SCT as the overarching framework and inductively for approaches identified from the interviews. RESULTS: All six main concepts of SCT (behavioral capacity, expectations, self-efficacy, observational learning, reinforcements, and reciprocal determinism) were observed in the three stages of symptom appraisal and help-seeking (detection, interpretation, and response) of patients with ARDs. While many participants reported that they were able and confident to detect their symptoms, they lacked the behavioral capacity and self-efficacy to interpret symptoms correctly, which resulted in delayed help-seeking and diagnosis. Possible approaches to address this suggested by participants (such as education of the general population) could improve behavioral capacity and self-efficacy in symptom interpretation and enhance expectations, observational learning, reinforcements, and reciprocal determinism in symptom response. CONCLUSION: Lack of behavioral capacity and self-efficacy was observed in symptom interpretation of patients with ARDs, which resulted in delayed help-seeking. Approaches could target the behavioral capacity and self-efficacy for symptom interpretation to facilitate early help-seeking and, in turn, earlier diagnosis among individuals with possible ARDs.

Symptom Appraisal and Help-Seeking Among Patients With Autoimmune Rheumatic Diseases: A Qualitative Study.

OBJECTIVE: Long diagnostic delay remains an unsolved problem in many autoimmune rheumatic diseases (ARDs). One of the major contributing factors is poor symptom appraisal and the resulting delays in help-seeking by patients themselves. We therefore aimed to understand the symptom appraisal and help-seeking experience among patients with ARDs in a multiethnic urban Asian population and to explore its influencing factors. METHODS: Semistructured interviews with 33 patients with ARDs were audio recorded and transcribed verbatim. We coded the transcripts deductively using the reported 3 stages of symptom appraisal (detection, interpretation, and response) as the framework, and inductively for newly emerging themes and subthemes. RESULTS: All 3 stages of the symptom appraisal and help-seeking journey (ie, symptom detection [by self and by others], symptom interpretation [causes, consequences, and required actions] and symptom response [no action, self-management, seeking help from nonhealthcare professionals, and seeking help from healthcare professionals]) were observed among patients. Interactions among these 3 stages were also observed: symptom interpretation was found to influence subsequent symptom detection, and the outcome of symptom response was found to influence both subsequent symptom detection and symptom interpretation. Various personal and socioenvironmental factors (eg, knowledge and cultural beliefs about the symptom) that influenced symptom appraisal and help-seeking were identified from the interviews. CONCLUSION: The symptom appraisal and help-seeking journey of patients with ARDs is an iterative process of detection, interpretation, and response, and is influenced by various personal and socioenvironmental factors. Addressing modifiable factors could shorten the symptom appraisal and help-seeking interval and improve patient outcomes.

Evaluation of Nailfold Capillaroscopy Online Training Using the Fast Track Algorithm.

OBJECTIVE: Nailfold capillaroscopy (NFC) is increasingly used in the early identification of systemic sclerosis (SSc)-related disorders. A consensus "Fast Track algorithm" was developed by the European Alliance of Associations for Rheumatology to aid differentiation of scleroderma from nonscleroderma pattern on NFC. Our objective was to evaluate the online training of NFC using the Fast Track algorithm in the assessment of scleroderma vs nonscleroderma NFC pattern. METHODS: Participants attended the NFC online training workshop and were taught the Fast Track algorithm. Following the training, participants independently evaluated 45 NFC images in the same session, and then 2 to 4 weeks later, through the online platform. Participants had to differentiate between scleroderma vs nonscleroderma pattern, and additionally nonscleroderma pattern (normal) vs nonscleroderma pattern (nonspecific). The inter- and intrarater Cohen [Formula: see text] agreement was calculated. RESULTS: Ninety-eight participants took part in the baseline evaluation, and 61 in the reevaluation session. For identification of scleroderma vs nonscleroderma pattern, the mean (95% CI) inter- and intrarater [Formula: see text] were 0.86 (0.83-0.88) and 0.83 (0.79-0.87), respectively. The overall inter- and intrarater [Formula: see text] in the identification of scleroderma, nonscleroderma (normal), and nonscleroderma (nonspecific) patterns were 0.71 (0.69-0.74) and 0.71 (0.67-0.75), respectively. For nonscleroderma (normal) vs nonscleroderma (nonspecific) pattern, the inter- and intrarater [Formula: see text] were 0.59 (0.55-0.63) and 0.59 (0.54-0.65), respectively. CONCLUSION: In this first study evaluating NFC online training using the Fast Track algorithm, we showed very good inter- and intrarater agreement for the identification of scleroderma and nonscleroderma NFC pattern, supporting the feasibility of online NFC standardized training workshops.

Approaches to improving symptom appraisal: a systematic literature review.

OBJECTIVES: Poor symptom appraisal (detection, interpretation and response to symptoms) plays a major role in prolonged prediagnosis interval in various health conditions. Theories and models have been proposed to study the symptom appraisal process but how they could be employed to improve symptom appraisal remains unclear. We therefore aimed to review approaches to improving symptom appraisal in the literature and to develop a theoretical framework that could guide the development of approaches to improving symptom appraisal among individuals in the general population. DESIGN: Systematic review. DATA SOURCES: Medline, Web of Science, PsycINFO, Embase, CINAHL and Scopus were searched from inception to 30 March 2021. ELIGIBILITY CRITERIA: We included original articles in English in which approaches to improve the detection, interpretation or response to symptoms for symptomatic individuals were described. We excluded articles in which approaches were developed to improve symptom appraisal among healthcare professionals. DATA EXTRACTION AND SYNTHESIS: A predefined data extraction form was used to extract the development, characteristics and evaluation of approaches to improving symptom appraisal. This formed the basis for the narrative synthesis. RESULTS: Of 19 046 publications identified from the literature search, 112 were selected for full-text review and 29 approaches comprising provision of knowledge of symptoms/signs and additional components (eg, symptom self-examination and comparison) for symptom appraisal were included in the synthesis. Less than half (41.4%) of these approaches were developed based on theories/models. Interestingly, despite the variety of theories/models adopted in developing these approaches, the components of these approaches were similar. CONCLUSION: Symptom appraisal is an essential process in a patient's journey that can be targeted to facilitate early diagnosis but is largely unstudied. Building on the literature, we proposed a theoretical framework and approaches to improving symptom appraisal. This could facilitate early identification of a variety of health conditions in the general population. TRIAL REGISTRATION NUMBER: CRD42021279500.

Work Productivity and Economic Burden of Systemic Sclerosis in a Multiethnic Asian Population.

OBJECTIVE: To assess work productivity, identify associated factors and evaluate the economic burden of systemic sclerosis (SSc) in a multiethnic Asian population. METHODS: Data on employment status and work productivity loss were collected. Associations between demographic and disease characteristics and unemployment status, work productivity loss, and activity impairment were examined using logistic and linear regression analyses, as appropriate. Costs of unemployment and work productivity loss were estimated using the human capital approach. RESULTS: Of 111 patients with a mean disease duration of 9.1 years, 33 (29.7%) were unemployed. Their mean age at unemployment was 44.2 years, equating to 22.8 years of lost employment. No demographic and disease characteristics were significantly associated with unemployment status in multivariable analysis. Of 73 employed patients, 39 (53.4%) reported work productivity loss, accounting for 45.9% of the working week. The presence of hyperlipidemia (coefficient -19.01, P = 0.03) was associated with work productivity loss in multivariable analysis. In total, 37 of 78 employed patients (47.4%) and 19 of 33 unemployed patients (57.6%) reported activity impairment, accounting for 42.2% and 50.0%, respectively, of the preceding week. The presence of hyperlipidemia (coefficient -18.56, P < 0.01) was associated with activity impairment in multivariable analysis. Annual cost of unemployment and work productivity loss were estimated to be $53,244 and $13,045 (Singapore dollar) per patient, respectively. CONCLUSION: SSc imposes significant unemployment and work productivity loss and causes a substantial economic burden to both affected individuals and society. Modifying the identified factors associated with unemployment and work productivity loss may reduce the burden of SSc.

Work disability in rheumatic diseases: Baseline results from an inception cohort.

AIM: We aimed to characterize work disability in patients with newly diagnosed rheumatic diseases and compare work characteristics between patients with rheumatic diseases and controls without rheumatic diseases at diagnosis. METHODS: Patients with inflammatory arthritis (IA) and osteoarthritis (OA), surrogates for autoimmune and non-autoimmune rheumatic diseases, respectively, and controls of working age were surveyed at diagnosis. Patients with rheumatic diseases who were employed before symptom onset were characterized as having work disability if they reported reduced work ability and productivity while remaining in the same job as before symptom onset, changed to a less demanding job or stopped working/retired. Work characteristics at diagnosis were compared between rheumatic diseases patients and controls. RESULTS: The unemployment rate before symptom onset was lower in patients with IA (20%) compared to patients with OA (32%). Among patients with IA and OA who were employed before symptom onset, 59% and 43% reported work disability, respectively (P = .04). The unemployment rate at diagnosis was comparable in patients with IA (26%) and higher in patients with OA (38%) compared to controls (29%). Employed patients with IA and OA, when compared with controls, reported poorer work ability (score: 37 vs 39 vs 41, P < .01; proportion with poor/moderate work ability: 48% vs 33% vs 21%, P < .01) and greater work productivity loss (score: 32 vs 29 vs 17, P < .01) at diagnosis. CONCLUSION: Rheumatic diseases impose significant work disability at diagnosis, highlighting the need for identification and interventions targeting work disability early in the course of disease.

Evaluation of Scleroderma Clinical Trials Consortium training recommendations on modified Rodnan skin score assessment in scleroderma.

AIM: The modified Rodnan skin score (mRSS) is a validated outcome measure for skin thickness in systemic sclerosis (SSc). Training has been shown to reduce variability in the measurement of mRSS. Our objective was to assess the inter- and intra-observer variability of mRSS scoring using the proposed recommendations for training by the Scleroderma Clinical Trials Consortium (SCTC) and World Scleroderma Foundation (WSF). METHOD: Fifty-two trainees and eight adult SSc patients participated in the SSc skin scoring workshop that was conducted in two sessions by four teachers. Each session, attended by 26 trainees, had a teaching and evaluation phase. The teaching phase comprised of: (a) lecture on mRSS scoring; (b) video demonstration of mRSS scoring; and (c) live demonstration of mRSS on one SSc patient. In the evaluation phase, each trainee independently assessed the mRSS in four SSc patients. For intra-observer reliability, 14 trainees re-assessed the mRSS of two SSc patients whom they had previously examined. We computed the inter- and intra-observer variability using a linear mixed model. RESULTS: For the evaluation phase, 34 (65.4%) trainees were within five units of the established teachers' score in 3 out of 4 patients. Overall, the whole group had acceptable inter-observer variability (intra-class correlation coefficient [ICC] = 0.71, mean = 8.64 and within-patient standard deviation [SD] = 4.25). The intra-observer ICC was 0.85 and within-patient SD was 2.73. CONCLUSION: There was good inter-observer and excellent intra-observer reliability. This is the first study examining the training of assessors using the SCTC/WSF recommendations and our results support the importance of standardized training for skin scoring.

A qualitative systematic review of the prevalence of coronary artery disease in systemic sclerosis.

AIMS: To review existing literature on the prevalence/incidence of coronary artery disease (CAD), and secondarily highlight risk factors for CAD in systemic sclerosis (SSc). METHODS: A PubMed and Cochrane Central Register of Controlled Trials search of studies (till 30 November 2013) relating to SSc and CAD was performed, retrieving 180 titles. INCLUSION CRITERIA: studies reporting CAD prevalence/incidence in SSc based on autopsy findings, coronary artery calcium scores, coronary angiographic findings and physician/patient-reported CAD. EXCLUSION CRITERIA: (i) not written in English; (ii) not concerned with human subjects; (iii) single case reports or review articles; (iv) genetic studies; and 95) other surrogate outcome measures of atherosclerosis. Quality assessment was done using the Newcastle-Ottawa score (range 0-9). RESULTS: Thirteen studies (Newcastle-Ottawa score 5-8) were selected. Of eight studies with controls, seven reported increased CAD prevalence (10-56%) or incidence (2.3%) compared to controls (prevalence 2-44%; incidence 1.5%). Of five studies without controls, CAD prevalence was 8-32%. Five of six studies reported that traditional cardiovascular risk factors were similar/reduced in SSc compared to controls. SSc was an independent risk factor for CAD, in addition to age (n = 2), hypercholesterolaemia (n = 3), male gender (n = 1), hypertension and diabetes (n = 1). Disease duration, renal involvement and pulmonary arterial hypertension were associated with CAD. CONCLUSIONS: Systemic sclerosis is associated with an increased prevalence/incidence of CAD. SSc is an independent risk factor for CAD. The association of CAD with SSc-related factors requires further research. Meanwhile, patients with SSc should be screened and treated for identified traditional cardiovascular risk factors.

Validation of the scleroderma health assessment questionnaire and quality of life in English and Chinese-speaking patients with systemic sclerosis.

AIMS: To perform cross-cultural adaptation and validation of the Scleroderma Health Assessment Questionnaire (S-HAQ) and Systemic Sclerosis Quality of Life scale (SSc-QoL) in English and Chinese-speaking patients with SSc. METHODS: In this Institutional Review Board approved study, patients seen at the rheumatology outpatient clinics were consecutively recruited over a 10-month period. We evaluated: (i) test-retest reliability using the intraclass correlation coefficient (ICC); (ii) known-groups construct validity by stratifying patients according to organ involvement and symptom severity; and (iii) convergent validity using Spearman's correlation with Short Form 36 version 2 (SF36v2). RESULTS: Forty-nine SSc patients completed the questionnaires: 26 Chinese, 23 English; 30 self-administered, 19 interviewer-administered, of which 35 responded to the retest. The SSc-QoL, HAQ Disability Index, intestinal Visual Analogue Scale (VAS), breathing VAS and overall disease severity VAS demonstrated high reliability (ICCs 0.71-0.93). The intestinal, finger ulcer and breathing S-HAQ VASs were able to differentiate patients according to their respective organ involvement. As hypothesized, the S-HAQ correlated better with the Physical Summary Component (PCS) than the Mental Summary Component (MCS) scores of the SF36v2. The SSc-QoL also demonstrated good convergent validity with the SF36v2 scales. CONCLUSION: In this pilot study, we provided preliminary evidence to demonstrate the good test-retest reliability and reasonable construct validity of S-HAQ and SSc-QoL for use in English and Chinese-speaking SSc patients. This forms the basis for future studies to assess more extensively their psychometric properties.

Evaluation of a new multi-parallel line immunoassay for systemic sclerosis-associated antibodies in an Asian population.

OBJECTIVES: To compare the SSc-line immunoassay (LIA) with conventional techniques of antibody detection, to evaluate its diagnostic utility and to describe clinical associations of antibodies in Asian SSc patients. METHODS: Stored sera from patients with SSc (n = 68), SLE (n = 49), OA (n = 41) and normal controls (NCs, n = 32) were evaluated. Cohen's κ and Bland-Altman plots were used to evaluate agreement. RESULTS: There was good agreement between LIA and ELISA for anti-Scl-70 (κ = 0.97), anti-CENPA (κ = 0.83), anti-CENPB (κ = 0.96) and anti-PmScl100 (κ = 1.00) (5.48-8.22% of values outside the 95% limits of agreement using Bland-Altman plots), and between LIA and IIF for anti-CENPA (κ = 0.81) and anti-CENPB (κ = 0.77) (P < 0.001). Using LIA, of 32 (32/68, 47%) SSc patients negative for anti-Scl-70 and anti-CENPA/B, 5 (5/32, 15%) were positive for anti-Ku, -Nor90, -fibrillarin and -RP155. Specificity of each antibody for SSc was at least 97% (vs OA/NC) and 94% (vs SLE), except for anti-Ro52 (63%). Anti-CENPB was associated with joint pain [odds ratio (OR) 0.17], interstitial lung disease (OR 0.24) and telangiectasia (OR 4.00) (P < 0.05). Anti-Ro60 was associated with pulmonary arterial hypertension (OR 3.89, P = 0.041). CONCLUSION: The SSc-LIA has good agreement with conventional techniques for selected antibodies and has good diagnostic utility.

Magnetic resonance imaging of the hand in systemic sclerosis.

OBJECTIVE: To evaluate the utility of magnetic resonance imaging (MRI) in systemic sclerosis (SSc)-associated arthropathy. METHODS: MRI of the hand was performed in patients presenting with joint pain/swelling in order (1) to determine the frequency of inflammation on MRI, and (2) to compare MRI with radiography. RESULTS: Of 17 patients with SSc, 10 (59%) had inflammatory MRI findings with synovitis (n = 8), erosions (n = 7), joint effusion (n = 7), or tenosynovitis (n = 8). Bone edema was present in 9 patients. Of 7 patients with MRI erosions, only 2 had radiographic erosions. CONCLUSION: Our study illustrates the usefulness of MRI in the accurate diagnosis and characterization of SSc-associated arthropathy.

Ethnic influence on disease manifestations and autoantibodies in Chinese-descent patients with systemic sclerosis.

OBJECTIVE: To investigate ethnic influence on disease manifestations and autoantibody profile in patients of Chinese descent with systemic sclerosis (SSc). METHODS: In a retrospective study of a multiethnic SSc cohort followed over a 17-year period, disease manifestations and autoantibody profile of patients of European and Chinese descent were compared. RESULTS: There were 300 patients of European descent and 36 of Chinese descent, with similar proportions of women (81% and 72%, respectively) and patients with diffuse SSc (50% and 56%). Patients of Chinese descent [mean age+/-standard deviation (SD) 52+/-16 yrs; p=0.05] were diagnosed at an older age compared to patients of European descent (mean+/-SD 46+/-12 yrs). Patients of Chinese descent compared to those of European descent had less frequent joint (69% vs 86%; p=0.01) and gastrointestinal involvement (78% vs 94%; p=0.004), but increased prevalence of myositis (17% vs 5%; p=0.01). Patients of Chinese descent had less frequent digital ulceration (36% vs 55%; p=0.04), and an absence of renal crisis. The frequency of cardiac and pulmonary involvement was similar in both groups. More patients of Chinese than of European descent were positive for anti-topoisomerase-I (47% vs 27%; p=0.02), anti-Ro (36% vs 10%; p=0.001), and anti-U1RNP (17% vs 5%; p=0.03) antibodies. The observed differences for anti-topoisomerase-I, anti-Ro, and joint and gastrointestinal manifestations persisted in the subgroup analysis of patients matched for sex, disease subtype, and age at diagnosis. CONCLUSION: Patients of Chinese descent have milder SSc disease with less frequent joint and gastrointestinal manifestations, less severe vasculopathy, but increased prevalence of myositis and certain autoantibodies. Research is needed to identify determinants (genetic, environmental, and cultural factors) of the relationship between ethnicity and disease.