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  • 1
    In: Rheumatology, Oxford University Press (OUP), Vol. 59, No. 12 ( 2020-12-01), p. 3900-3905
    Kurzfassung: To determine the frequency of self-reported occupational exposure to silica in SSc patients enrolled in the Australian Scleroderma Cohort Study, and to compare the disease characteristics of the silica-exposed patients with those of the non-exposed patients. Method Data collected over a 12-year period from 1670 SSc patients were analysed. We compared the demographic and clinical characteristics of those who reported occupational silica exposure with those who did not. A subgroup analysis of male patients was performed, as well as a multivariable analysis of correlates of silica exposure. Results Overall, 126 (7.5%) of the cohort reported occupational silica exposure. These individuals were more likely to be male (73 of 231, i.e. 31.6% males exposed) and to have worked in mining and construction industries. Those who reported silica exposure were younger at the onset of SSc skin involvement [odds ratio (OR) 0.9, P = 0.02], of male gender (OR 14.9, P  & lt; 0.001), have joint contractures (OR 1.8, P = 0.05) and have higher physical disability as defined by scleroderma HAQ (OR 1.4, P = 0.01). Conclusion The highest percentage of silica exposure was found in males. These patients were more likely to have the presence of certain clinical manifestations and Scl-70 antibody, which is known to confer a poor prognosis. These findings support the association between occupational silica exposure and the subsequent development of SSc. Further investigation is required to describe the range of clinical manifestations and disease course, including prognosis and treatment response, in those diagnosed with occupationally induced SSc compared with idiopathic SSc.
    Materialart: Online-Ressource
    ISSN: 1462-0324 , 1462-0332
    Sprache: Englisch
    Verlag: Oxford University Press (OUP)
    Publikationsdatum: 2020
    ZDB Id: 1474143-X
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 2
    In: Arthritis Research & Therapy, Springer Science and Business Media LLC, Vol. 25, No. 1 ( 2023-05-12)
    Kurzfassung: To describe the clinical phenotype and prognosis of people in the Australian Scleroderma (SSc) Cohort Study with pulmonary arterial hypertension (PAH) with or without interstitial lung disease (ILD). Methods Participants meeting ACR/EULAR criteria for SSc were divided into four mutually exclusive groups: those meeting criteria for PAH (PAH-only), ILD (ILD-only), concurrent PAH and ILD (PAH-ILD) or neither PAH nor ILD (SSc-only). Logistic or linear regression analyses were used for associations between clinical features, health-related quality of life (HRQoL) and physical function. Survival analysis was performed using Kaplan–Meier estimates and Cox-regression modelling. Results Of 1561 participants, 7% fulfilled criteria for PAH-only, 24% ILD-only, 7% PAH-ILD and 62% SSc-only. People with PAH-ILD were more frequently male, with diffuse skin involvement, higher inflammatory markers, older age of SSc onset and higher frequency of extensive ILD than the cohort overall ( p   〈  0.001). People of Asian race more frequently developed PAH-ILD ( p   〈  0.001). People with PAH-ILD or PAH-only had worse WHO functional class and 6-min-walk-distance than ILD-only ( p   〈  0.001). HRQoL scores were worst in those with PAH-ILD ( p   〈  0.001). Survival was reduced in the PAH-only and PAH-ILD groups ( p   〈  0.01). Multivariable hazard modelling demonstrated the worst prognosis in extensive ILD and PAH (HR = 5.65 95% CI 3.50–9.12 p   〈  0.01), followed by PAH-only (HR = 4.21 95% CI 2.89–6.13 p   〈  0.01) and PAH with limited ILD (HR = 2.46 95% CI 1.52–3.99 p   〈  0.01). Conclusions The prevalence of concurrent PAH-ILD in the ASCS is 7%, with poorer survival in those patients with PAH-ILD compared to ILD or SSc alone. The presence of PAH confers a poorer overall prognosis than even extensive ILD; however, further data are required to better understand the clinical outcomes of this high-risk patient group.
    Materialart: Online-Ressource
    ISSN: 1478-6362
    Sprache: Englisch
    Verlag: Springer Science and Business Media LLC
    Publikationsdatum: 2023
    ZDB Id: 2041668-4
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 3
    In: Rheumatology, Oxford University Press (OUP), Vol. 62, No. 9 ( 2023-09-01), p. 3059-3066
    Kurzfassung: Damage accrual in SSc can be tracked using the Scleroderma Clinical Trials Consortium Damage Index (DI). Our goal was to develop a prediction model for damage accrual in SSc patients with early disease. Methods Using patients with & lt;2 years disease duration from Canada and Australia as a derivation cohort, and from the Netherlands as a validation cohort, we used group-based trajectory modelling (GBTM) to determine ‘good’ and ‘bad’ latent damage trajectories. We developed a prediction model from this analysis and applied it to patients from derivation and validation cohorts. We plotted the actual DI trajectories of the patients predicted to be in ‘good’ or ‘bad’ groups. Results We found that the actual trajectories of damage accumulation for lcSSc and dcSSc were very different, so we studied each subset separately. GBTM found two distinct trajectories in lcSSc and three in dcSSc. We collapsed the two worse trajectories in the dcSSc into one group and developed a prediction model for inclusion in either ‘good’ or ‘bad’ trajectories. The performance of models using only baseline DI and sex was excellent with ROC AUC of 0.9313 for lcSSc and 0.9027 for dcSSc. Using this model, we determined whether patients would fall into ‘good’ or ‘bad’ trajectory groups and then plotted their actual trajectories which showed clear differences between the predicted ‘good’ and ‘bad’ cases in both derivation and validation cohorts. Conclusions A simple model using only cutaneous subset, baseline DI and sex can predict damage accumulation in early SSc.
    Materialart: Online-Ressource
    ISSN: 1462-0324 , 1462-0332
    Sprache: Englisch
    Verlag: Oxford University Press (OUP)
    Publikationsdatum: 2023
    ZDB Id: 1474143-X
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 4
    In: Arthritis Care & Research, Wiley, Vol. 72, No. 11 ( 2020-11), p. 1625-1635
    Kurzfassung: 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).
    Materialart: Online-Ressource
    ISSN: 2151-464X , 2151-4658
    URL: Issue
    RVK:
    Sprache: Englisch
    Verlag: Wiley
    Publikationsdatum: 2020
    ZDB Id: 2016713-1
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 5
    In: Arthritis Care & Research, Wiley, Vol. 73, No. 5 ( 2021-05), p. 732-741
    Kurzfassung: 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.
    Materialart: Online-Ressource
    ISSN: 2151-464X , 2151-4658
    URL: Issue
    RVK:
    Sprache: Englisch
    Verlag: Wiley
    Publikationsdatum: 2021
    ZDB Id: 2016713-1
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 6
    In: BMC Pulmonary Medicine, Springer Science and Business Media LLC, Vol. 19, No. 1 ( 2019-12)
    Kurzfassung: To quantify the financial cost of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc). Methods Healthcare use was captured through data linkage, wherein clinical data for SSc patients enrolled in the Australian Scleroderma Cohort Study were linked with hospital, emergency department (ED) and ambulatory care databases (MBS) for the period 2008–2015. PAH was diagnosed on right heart catheter according to international criteria. Determinants of healthcare cost were estimated using logistic regression. Results Total median (25th–75th) healthcare cost per patient (including hospital, ED and MBS cost but excluding medication cost) for our cohort during 2008–2015 was AUD$37,685 (18,144-78,811) with an annual per patient healthcare cost of AUD$7506 (5273-10,654). Total healthcare cost was higher for SSc-PAH patients compared with those without PAH with a total cost per patient of AUD$70,034 (37,222-110,814) vs AUD$34,325 (16,093 – 69,957), p   〈  0.001 respectively with an annual excess healthcare cost per PAH patient of AUD$2463 (1973-1885), p   〈  0.001. The cost of SSc-PAH occurs early post PAH diagnosis with 89.4% utilizing a healthcare service within the first 12 months post PAH diagnosis with an associated cost per patient of AUD$4125 (0–15,666). PAH severity was the main significant determinant of increased healthcare cost (OR 2.5, p  = 0.03) in our PAH cohort. Conclusions Despite SSc-PAH being a low prevalence disease, it is associated with significant healthcare resource utilization and associated economic burden, predominantly driven by the severity of PAH.
    Materialart: Online-Ressource
    ISSN: 1471-2466
    Sprache: Englisch
    Verlag: Springer Science and Business Media LLC
    Publikationsdatum: 2019
    ZDB Id: 2059871-3
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 7
    In: Arthritis Research & Therapy, Springer Science and Business Media LLC, Vol. 21, No. 1 ( 2019-12)
    Kurzfassung: To determine the frequency and clinical characteristics of systemic sclerosis-related digital ulcers, and associated direct health care costs, quality of life, and survival. Methods Digital ulcers (DUs) were defined as an area with a visually discernible depth and a loss of continuity of epithelial coverage. DU severity was calculated based on the physician reported highest number of new DUs at clinical review (mild = 1–5 DUs, moderate 6–10 DUs, severe 〉  10 DUs). Healthcare use was captured through data linkage, wherein SSc clinical data captured prospectively in a dedicated clinical database were linked with health services databases to capture hospital admissions, emergency department (ED) presentations and ambulatory care (MBS) utilization and cost for the period 2008–2015. Healthcare cost determinants were estimated using logistic regression. Results Among 1085 SSc patients, 48.6% experienced a DU over a mean follow-up of 5.2 ± 2.5 years. Those who developed DUs were more likely to have diffuse disease subtype (34.9% vs 18.2%, p   〈  0.001), anti-Scl-70 antibody (18.9% vs 9.3%, p   〈  0.001), and a younger age at SSc onset (43.6 ± 13.9 vs 48.8 ± 14.0 years, p   〈  0.001) in addition to reduced health-related quality of life (HRQoL) measured by the SF-36 but without a significant impact on survival. SSc patients with a history of a DU utilized significantly more healthcare resources per annum than those without a DU, including hospitalizations, ED presentation, and ambulatory care services. Total healthcare services, excluding medications, were associated with an annual excess cost per DU patient of AUD$12,474 (8574-25,677), p   〈  0.001, driven by hospital admission and ED presentation costs. Conclusion DUs place a large burden on the patient and healthcare system through reduced HRQoL and increased healthcare resource utilization and associated cost.
    Materialart: Online-Ressource
    ISSN: 1478-6362
    Sprache: Englisch
    Verlag: Springer Science and Business Media LLC
    Publikationsdatum: 2019
    ZDB Id: 2041668-4
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 8
    In: Rheumatology, Oxford University Press (OUP), Vol. 59, No. 8 ( 2020-08-01), p. 1878-1888
    Kurzfassung: To quantify the burden of interstitial lung disease (ILD) in SSc. Methods Clinical data for SSc patients enrolled in the Australian Scleroderma Cohort Study were linked with healthcare databases for the period 2008–2015. ILD was defined by characteristic fibrotic changes on high-resolution CT (HRCT) lung, while severity was defined by the extent lung involvement on HRCT (mild & lt;10%, moderate 10–30%, severe & gt;30%). Determinants of healthcare cost were estimated using logistic regression. Results SSc-ILD patients utilized more healthcare resources, including hospitalization, emergency department presentation and ambulatory care services, than those without ILD with a total cost per patient of AUD$48 368 (26 230–93 615) vs AUD$33 657 (15 144–66 905), P & lt;0.001) between 2008–2015. Healthcare utilization was associated with an annual median (25th–75th) excess cost per SSc-ILD patient compared with those without ILD of AUD$1192 (807–1212), P & lt;0.001. Increasing ILD severity was associated with significantly more healthcare utilization and costs with an annual excess cost per patient with severe ILD compared with mild ILD of AUD$2321 (645–1846), P & lt;0.001. ILD severity and the presence of coexistent PAH were the main determinants of overall healthcare cost above median for this SSc-ILD cohort (OR 5.1, P & lt;0.001, and OR 2.6, P=0.01, respectively). Furthermore, SSc-ILD patients reported worse physical HRQoL compared with those without ILD [34.3 (10.5) vs 39.1 (10.8), P & lt;0.001], with a progressive decline with increasing ILD severity (P=0.002). Conclusion SSc-ILD places a large burden on the healthcare system and the patient through poor HRQoL in addition to incremental healthcare resource utilization and associated direct cost.
    Materialart: Online-Ressource
    ISSN: 1462-0324 , 1462-0332
    Sprache: Englisch
    Verlag: Oxford University Press (OUP)
    Publikationsdatum: 2020
    ZDB Id: 1474143-X
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 9
    In: Medicine, Ovid Technologies (Wolters Kluwer Health), Vol. 96, No. 48 ( 2017-12), p. e8503-
    Materialart: Online-Ressource
    ISSN: 0025-7974
    Sprache: Englisch
    Verlag: Ovid Technologies (Wolters Kluwer Health)
    Publikationsdatum: 2017
    ZDB Id: 2049818-4
    Standort Signatur Einschränkungen Verfügbarkeit
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  • 10
    In: The Journal of Rheumatology, The Journal of Rheumatology, Vol. 50, No. 4 ( 2023-04), p. 519-525
    Kurzfassung: 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.
    Materialart: Online-Ressource
    ISSN: 0315-162X , 1499-2752
    RVK:
    Sprache: Englisch
    Verlag: The Journal of Rheumatology
    Publikationsdatum: 2023
    Standort Signatur Einschränkungen Verfügbarkeit
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