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  • 1
    Online Resource
    Online Resource
    Occupational silica exposure in an Australian systemic sclerosis cohort
    Oxford University Press (OUP) ; 2020
    In:  Rheumatology Vol. 59, No. 12 ( 2020-12-01), p. 3900-3905
    Details
    In: Rheumatology, Oxford University Press (OUP), Vol. 59, No. 12 ( 2020-12-01), p. 3900-3905
    Abstract: 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.
    Type of Medium: Online Resource
    ISSN: 1462-0324 , 1462-0332
    URL: Article
    DOI: 10.1093/rheumatology/keaa446
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2020
    detail.hit.zdb_id: 1474143-X
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  • 2
    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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  • 3
    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
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  • 4
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    Online Resource
    Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis
    Springer Science and Business Media LLC ; 2023
    In:  Arthritis Research & Therapy Vol. 25, No. 1 ( 2023-05-12)
    Details
    In: Arthritis Research & Therapy, Springer Science and Business Media LLC, Vol. 25, No. 1 ( 2023-05-12)
    Abstract: 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.
    Type of Medium: Online Resource
    ISSN: 1478-6362
    URL: Article
    DOI: 10.1186/s13075-023-03059-x
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2023
    detail.hit.zdb_id: 2041668-4
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  • 5
    Online Resource
    Online Resource
    The economic burden of systemic sclerosis related pulmonary arterial hypertension in Australia
    Springer Science and Business Media LLC ; 2019
    In:  BMC Pulmonary Medicine Vol. 19, No. 1 ( 2019-12)
    Details
    In: BMC Pulmonary Medicine, Springer Science and Business Media LLC, Vol. 19, No. 1 ( 2019-12)
    Abstract: 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.
    Type of Medium: Online Resource
    ISSN: 1471-2466
    URL: Article
    DOI: 10.1186/s12890-019-0989-1
    Language: English
    Publisher: Springer Science and Business Media LLC
    Publication Date: 2019
    detail.hit.zdb_id: 2059871-3
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  • 6
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    Online Resource
    The clinical and economic burden of systemic sclerosis related interstitial lung disease
    Oxford University Press (OUP) ; 2020
    In:  Rheumatology Vol. 59, No. 8 ( 2020-08-01), p. 1878-1888
    Details
    In: Rheumatology, Oxford University Press (OUP), Vol. 59, No. 8 ( 2020-08-01), p. 1878-1888
    Abstract: 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.
    Type of Medium: Online Resource
    ISSN: 1462-0324 , 1462-0332
    URL: Article
    DOI: 10.1093/rheumatology/kez532
    Language: English
    Publisher: Oxford University Press (OUP)
    Publication Date: 2020
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  • 7
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    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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