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  • 1
    Online Resource
    Online Resource
    Clinical Features of Systemic Sclerosis–Mixed Connective Tissue Disease and Systemic Sclerosis Overlap Syndromes
    Wiley ; 2021
    In:  Arthritis Care & Research Vol. 73, No. 5 ( 2021-05), p. 732-741
    Details
    In: Arthritis Care & Research, Wiley, Vol. 73, No. 5 ( 2021-05), p. 732-741
    Abstract: To describe the clinical characteristics and outcomes of systemic sclerosis–mixed connective tissue disease (SSc–MCTD) and SSc overlap syndrome. Methods We included patients from the Australian Scleroderma Cohort Study who met American College of Rheumatology/European Alliance of Associations for Rheumatology criteria for SSc. Three mutually exclusive groups were created: SSc–MCTD, SSc overlap, and SSc only. Univariate comparison of clinical features was performed by analysis of variance or chi‐square test. Survival analysis was performed using Kaplan‐Meier (KM) curves and Cox proportional hazards regression models. Results Of 1,728 patients, 97 (5.6%) had SSc–MCTD, and 126 (7.3%) had SSc overlap. Those with MCTD–SSc were more commonly Asian (18.3% versus 10.1% in SSc overlap, and 3.6% in SSc only; P 〈 0.0001) and younger at disease onset (38.4 years versus 46.5 or 46.8 years, P 〈 0.0001). Those with SSc–MCTD or SSc overlap were more likely to have limited cutaneous SSc. All 3 groups had similar frequency of interstitial lung disease (ILD), although pulmonary arterial hypertension (PAH) was less common in SSc overlap. Synovitis and myositis were more common in SSc overlap and SSc–MCTD than in SSc only. KM curves showed better survival in SSc–MCTD than SSc overlap or SSc only ( P = 0.011), but this was not significant after adjustment for sex and age at disease onset. SSc‐specific antibodies were survival prognostic markers, with antinuclear antibody centromere or anti‐RNP conferring better survival than anti–Scl‐70 or anti–RNA polymerase III ( P = 0.005). Patients with SSc–MCTD and SSc overlap had lower mortality following diagnosis of ILD and PAH than patients with SSc only. Conclusion This study provides insights into the clinical characteristics of patients with SSc–MCTD, SSc overlap, and SSc only and shows that anti‐RNP antibodies are associated with better survival than anti–Scl‐70 and anti‐RNA polymerase III antibodies.
    Type of Medium: Online Resource
    ISSN: 2151-464X , 2151-4658
    URL: Issue
    URL: Article
    DOI: 10.1002/acr.v73.5
    DOI: 10.1002/acr.24167
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2021
    detail.hit.zdb_id: 2016713-1
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  • 2
    Online Resource
    Online Resource
    Incidence, Risk Factors, and Outcomes of Cancer in Systemic Sclerosis
    Wiley ; 2020
    In:  Arthritis Care & Research Vol. 72, No. 11 ( 2020-11), p. 1625-1635
    Details
    In: Arthritis Care & Research, Wiley, Vol. 72, No. 11 ( 2020-11), p. 1625-1635
    Abstract: To quantify the burden of cancer in systemic sclerosis (SSc). Methods Standardized incidence ratios (SIRs) and standardized mortality ratios relative to the general Australian population were derived. Cox proportional hazards regression was used to estimate survival in patients with SSc with cancer compared to patients without. Determinants of cancer were identified using logistic regression. Health care cost was quantified through cross‐jurisdictional data linkage. Results This SSc cohort of 1,727 had a cancer incidence of 1.3% per year and a prevalence of 14.2%, with a SIR of 2.15 (95% confidence interval [95% CI] 1.84–2.49). The most common cancers were breast, melanoma, hematologic, and lung. Anti–RNA polymerase III (RNAP) antibody was associated with an increased risk of cancer (odds ratio [OR] 2.9, P = 0.044), diagnosed within 5 years of SSc disease onset. Calcium channel blockers were associated with a higher risk of overall cancer (OR 1.47, P = 0.016), breast cancer (OR 1.61, P = 0.051), and melanoma (OR 2.01, P = 0.042). Interstitial lung disease (ILD) was associated with lung cancer (OR 2.83, P = 0.031). Incident SSc cancer patients had 〉 2‐fold increased mortality compared to patients with SSc without cancer (hazard ratio 2.85 [95% CI 1.51–5.37], P = 0.001). Patients with SSc and cancer utilized more health care than those without cancer, with an excess annual health care cost of $1,496 Australian ( P 〈 0.001). Conclusion SSc carries an increased risk of developing cancer, particularly lung cancer associated with ILD, and breast cancer and melanoma occurring close to SSc disease onset in association with RNAP antibodies. Compared to those patients without cancer, patients with SSc and cancer had higher mortality and an increased health care cost, with an annual excess per patient cost of $1,496 Australian ( P 〈 0.001).
    Type of Medium: Online Resource
    ISSN: 2151-464X , 2151-4658
    URL: Issue
    URL: Article
    DOI: 10.1002/acr.v72.11
    DOI: 10.1002/acr.24076
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2020
    detail.hit.zdb_id: 2016713-1
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  • 3
    Online Resource
    Online Resource
    Evaluation of Patient and Physician Assessments of Gastrointestinal Disease Activity in Systemic Sclerosis
    The Journal of Rheumatology ; 2023
    In:  The Journal of Rheumatology Vol. 50, No. 4 ( 2023-04), p. 519-525
    Details
    In: The Journal of Rheumatology, The Journal of Rheumatology, Vol. 50, No. 4 ( 2023-04), p. 519-525
    Abstract: To assess whether patient and physician global assessment of gastrointestinal tract (GIT) disease in systemic sclerosis (SSc) are associated with a meaningful change in disease status. Methods One hundred forty-three participants from the Australian Scleroderma Cohort Study were recruited to this study. Using logistic regression analysis, we evaluated the relationship between patient-reported and physician-assessed GIT disease status and symptoms, measures of health-related quality of life (36-item Short Form Health Survey [SF-36]) and GIT disease severity, measured by th e Scleroderma Clinical Trials Consortium UCLA Gastrointestinal Tract 2.0 (GIT 2.0) score. Results Patient-reported worsening of GIT symptoms in the month preceding assessment was significantly associated with more severe GIT disease (odds ratio [OR] 6.14, P 〈 0.01) and progressive worsening GIT disease severity as measured by the GIT 2.0 score (OR 45.98, P 〈 0.01). The new onset of reflux was the only specific symptom associated with patient-reported GIT disease activity (OR 2.98, P = 0.04). Physician-assessed GIT disease activity was not significantly associated with higher GIT 2.0 scores or increasing severity of disease. Patient-reported and physician-assessed GIT activity was not associated with SF-36 scores. Conclusion In the absence of objective measures of GIT disease activity in SSc, patient-reported symptoms of GIT disease could be used to indicate disease activity and should merit consideration for inclusion in a multisystem disease activity index.
    Type of Medium: Online Resource
    ISSN: 0315-162X , 1499-2752
    URL: Article
    DOI: 10.3899/jrheum.220832
    RVK:
    XA 10000
    Language: English
    Publisher: The Journal of Rheumatology
    Publication Date: 2023
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  • 4
    Online Resource
    Online Resource
    Evaluation of the European Society of Cardiology Risk Assessment Score in Incident Systemic Sclerosis‐Associated Pulmonary Arterial Hypertension
    Wiley ; 2024
    In:  Arthritis Care & Research Vol. 76, No. 7 ( 2024-07), p. 973-983
    Details
    In: Arthritis Care & Research, Wiley, Vol. 76, No. 7 ( 2024-07), p. 973-983
    Abstract: 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.
    Type of Medium: Online Resource
    ISSN: 2151-464X , 2151-4658
    URL: Issue
    URL: Article
    DOI: 10.1002/acr.v76.7
    DOI: 10.1002/acr.25328
    RVK:
    XA 30325
    Language: English
    Publisher: Wiley
    Publication Date: 2024
    detail.hit.zdb_id: 2016713-1
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  • 5
    Online Resource
    Online Resource
    Development and validation of the Scleroderma Clinical Trials Consortium Damage Index (SCTC-DI): a novel instrument to quantify organ damage in systemic sclerosis
    BMJ ; 2019
    In:  Annals of the Rheumatic Diseases Vol. 78, No. 6 ( 2019-06), p. 807-816
    Details
    In: Annals of the Rheumatic Diseases, BMJ, Vol. 78, No. 6 ( 2019-06), p. 807-816
    Abstract: We sought to develop the first Damage Index (DI) in systemic sclerosis (SSc). Methods The conceptual definition of ‘damage’ in SSc was determined through consensus by a working group of the Scleroderma Clinical Trials Consortium (SCTC). Systematic literature review and consultation with patient partners and non-rheumatologist experts produced a list of potential items for inclusion in the DI. These steps were used to reduce the items: (1) Expert members of the SCTC (n=331) were invited to rate the appropriateness of each item for inclusion, using a web-based survey. Items with 〉 60% consensus were retained; (2) Using a prospectively acquired Australian cohort data set of 1568 patients, the univariable relationships between the remaining items and the endpoints of mortality and morbidity (Physical Component Summary score of the Short Form 36) were analysed, and items with p 〈 0.10 were retained; (3) using multivariable regression analysis, coefficients were used to determine a weighted score for each item. The DI was externally validated in a Canadian cohort. Results Ninety-three (28.1%) complete survey responses were analysed; 58 of 83 items were retained. The univariable relationships with death and/or morbidity endpoints were statistically significant for 22 items, with one additional item forced into the multivariable model by experts due to clinical importance, to create a 23-item weighted SCTC DI (SCTC-DI). The SCTC-DI was predictive of morbidity and mortality in the external cohort. Conclusions Through the combined use of consensus and data-driven methods, a 23-item SCTC-DI was developed and retrospectively validated.
    Type of Medium: Online Resource
    ISSN: 0003-4967 , 1468-2060
    URL: Article
    DOI: 10.1136/annrheumdis-2018-214764
    RVK:
    XA 10000
    Language: English
    Publisher: BMJ
    Publication Date: 2019
    detail.hit.zdb_id: 1481557-6
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