Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVES: We established a multicentre prospective registry of patients with systemic sclerosis (SSc) in Japan to evaluate the outcomes in the modern treatment era. This report presents the baseline characteristics of patients enrolled in the registry. METHODS: Adult SSc patients were prospectively enrolled from 20 medical centres across Japan. Baseline data, including demographics, organ involvement, autoantibody profiles, and patient-reported outcomes, were collected using a dedicated electronic data capture system. RESULTS: A total of 835 patients were eligible for analysis. The cohort was predominantly female (85.1%), with a median age of 64 years at enrolment, and 35.0% had diffuse cutaneous SSc. Autoantibodies included anticentromere (38.9%), anti-topoisomerase I (topo I; 33.5%), anti-RNA polymerase III (RNAP III; 12.4%), and anti-U1 RNP (12.9%). Interstitial lung disease (ILD) was the most common organ manifestation (56.0%), followed by upper gastrointestinal (GI) involvement (42.5%), heart involvement (5.3%), pulmonary hypertension (3.4%), scleroderma renal crisis (1.9%), and lower GI involvement (1.2%). Patients with anti-topo I had the worst patient global assessment, whereas those with anti-RNAP III patients had the worst physician global assessment. CONCLUSIONS: This prospective registry captures real-world data on SSc patients, providing a valuable resource for understanding the clinical spectrum and outcomes in contemporary practice in Japan.

DOI： 10.1093/mr/roaf109

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Language：English   Publishing type：Research paper (scientific journal)  

OBJECTIVE: To evaluate clinical and epidemiological features of juvenile-onset systemic sclerosis (jSSc) in Japan and to identify racial and generational differences. METHODS: We surveyed patients with jSSc (developed before the age of 18 years) who visited selected facilities in Japan between January 2016 and December 2020. We estimated the number of patients with jSSc and the annual incidence rate in Japan. Thereafter, differences in clinical characteristics by disease subtype, autoantibody, and age at investigation were analyzed and compared with previous cohorts. RESULTS: Of the 3,005 institutions selected for the first survey, 1,845 (61.8%) responded. The estimated number of patients with jSSc was 299, while the estimated annual incidence rate ranged from 0.98 to1.59 per 1,000,000 children (aged <18 years) from 2016 to 2020. In the second-stage survey, 130 cases were analyzed, of which 85 (65.4%) had diffuse cutaneous SSc (dcSSc), 77.7% were female, while the median ages at onset and survey was 11 and 21 years, respectively. Autoantibody positivity was 62.4% for anti-topoisomerase I antibody (ATA) and 12.9% for anti-centromere antibody, while anti-PM-Scl antibody was very rare. In total, interstitial lung disease was present in 40.8% of patients (predominantly dcSSc and ATA positive), gastrointestinal lesions in 36.9%, pulmonary arterial hypertension in 7.7%, and no renal crisis. CONCLUSION: This is the largest national survey of jSSc characteristics analyzed in detail by autoantibody and disease subtype. Japanese jSSc was characterized by very high ATA-positivity rate. However, the frequency of major organ involvement was similar to previous reports of jSSc in the west.

DOI： 10.3899/jrheum.2025-0175

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BACKGROUND: Systemic sclerosis (SSc) primary heart involvement (SSc-pHI) is one of the leading causes of mortality in SSc. We aimed to evaluate risk factors for SSc-pHI and its progression and the outcomes in the EUSTAR (European Scleroderma Trials and Research) cohort. METHODS: SSc-pHI was defined according to the World Scleroderma Foundation/Heart Failure Association definition. Data from 5741 patients with SSc in the EUSTAR cohort were analyzed. Additional cardiovascular data were collected from a subcohort of 838 patients with SSc. Lasso regression was used for risk factor analyses. Kaplan-Meier estimator was used for survival analyses. Progression of SSc-pHI was evaluated by a study definition developed by rheumatology and cardiology experts. RESULTS: Risk factors for the presence of SSc-pHI comprised skeletal muscle atrophy (odds ratio [OR], 2.00 [95% CI, 1.00-2.68]), age (OR, 1.91 [95% CI, 1.73-2.03]), male sex (OR, 1.77 [95% CI, 1.42-2.05]), swollen joints (OR, 1.70 [95% CI, 1.47-1.98]), skeletal muscle weakness (OR, 1.38 [95% CI, 1.00-1.85]), and tendon friction rubs (OR, 1.46 [95% CI, 1.00-1.77]) (n=3276). Telangiectasia (OR, 2.10 [95% CI, 1.38-2.72]), intestinal symptoms (OR, 1.70 [95% CI, 1.04-2.42]), age (OR, 1.47 [95% CI, 1.21-1.62]), and antitopoisomerase I antibodies (OR, 1.37 [95% CI, 1.00-1.77]) were associated with an increased risk for new onset of SSc-pHI (n=1000). Survival rate was significantly lower in patients with SSc-pHI than in those without (P value <0.0001, n=3768). Patients with SSc-pHI had a lower survival rate than patients with interstitial lung disease (n=3365). Swollen joints were associated with an increased risk of progressive SSc-pHI (OR, 2.49 [95% CI, 1.79-3.52]) (n=595). Tendon friction rubs (OR, 1.21 [95% CI, 0.94-1.90]) increased the risk of heart failure with preserved ejection fraction in patients with SSc-pHI. CONCLUSIONS: We defined progressive SSc-pHI and identified risk factors for new onset and progression of SSc-pHI and for SSc-pHI-associated heart failure with preserved ejection fraction in the largest cohort with SSc. These findings may guide patient stratification for diagnostic workup and therapy.

DOI： 10.1161/JAHA.124.036730

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DOI： 10.1111/1756-185X.70017

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INTRODUCTION: Heart involvement is a common problem in systemic sclerosis. Recently, a definition of systemic sclerosis primary heart involvement had been proposed. Our aim was to establish consensus guidance on the screening, diagnosis and follow-up of systemic sclerosis primary heart involvement patients. METHODS: A systematic literature review was performed to investigate the tests used to evaluate heart involvement in systemic sclerosis. The extracted data were categorized into relevant domains (conventional radiology, electrocardiography, echocardiography, cardiac magnetic resonance imaging, laboratory, and others) and presented to experts and one patient research partner, who discussed the data and added their opinion. This led to the formulation of overarching principles and guidance statements, then reviewed and voted on for agreement. Consensus was attained when the mean agreement was ⩾7/10 and of ⩾70% of voters. RESULTS: Among 2650 publications, 168 met eligibility criteria; the data extracted were discussed over three meetings. Seven overarching principles and 10 guidance points were created, revised and voted on. The consensus highlighted the importance of patient counseling, differential diagnosis and multidisciplinary team management, as well as defining screening and diagnostic approaches. The initial core evaluation should integrate history, physical examination, rest electrocardiography, trans-thoracic echocardiography and standard serum cardiac biomarkers. Further investigations should be individually tailored and decided through a multidisciplinary management. The overall mean agreement was 9.1/10, with mean 93% of experts voting above 7/10. CONCLUSION: This consensus-based guidance on screening, diagnosis and follow-up of systemic sclerosis primary heart involvement provides a foundation for standard of care and future feasibility studies that are ongoing to support its application in clinical practice.

DOI： 10.1177/23971983231163413

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Language：Japanese   Publisher：(一社)日本乳癌学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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Language：Japanese   Publisher：日本内科学会-関東地方会  

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Language：Japanese   Publisher：(一社)日本リウマチ学会  

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