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OBJECTIVES: The gastrointestinal tract (GIT) is the most commonly affected internal organ in systemic sclerosis (SSc). We sought to determine the prevalence and impact of GIT symptoms on survival and patient-reported outcomes. METHODS: 907 consecutive patients from the Australian Scleroderma Cohort Study (ASCS) who had prospectively completed the University of California Los Angeles Scleroderma Clinical Trials Consortium Gastrointestinal Tract 2.0 questionnaire (UCLA GIT) between 2015 and 2021 were included. The association between UCLA GIT scores and physical function (SHAQ), QoL (SF-36), mood (PROMIS anxiety and depression domains), fatigue (FACIT-fatigue score) and employment was investigated using multivariable population-averaged panel models using Generalized Estimating Equations (GEE). Kaplan-Meier curves and multivariable Cox proportional hazard regression model were used to evaluate survival according to total UCLA GIT scores. RESULTS: GIT symptoms were reported in 87% of participants with 46-52% reporting moderate to very severe symptoms of reflux, distension, diarrhoea and constipation. Higher total UCLA GIT scores were associated with worse QoL, physical function, fatigue, anxiety and depression (p<0.001). In multivariable GEE analysis, moderate and severe to very severe total scores, reflux and distension scores were associated with worse physical function, QoL, fatigue, anxiety and depression compared to those with mild scores (p<0.05). Patients with severe total scores and diarrhoea scores were more likely to be unemployed compared to those with mild scores (p<0.05). UCLA GIT total scores were not independently associated with mortality in our cohort. CONCLUSION: GIT manifestations are common in SSc and negatively impact QoL, physical function and employment but are not directly associated with increased mortality.

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BACKGROUND: To determine the relationship between gastroesophageal reflux disease (GORD) and its treatment and interstitial lung disease in patients with systemic sclerosis (SSc). METHODS: SSc patients from the Australian Scleroderma Cohort Study (ASCS) were included. GORD was defined as self-reported GORD symptoms, therapy with a proton pump inhibitor (PPI) or histamine 2 receptor antagonist (H2RA) and/or the presence of reflux oesophagitis diagnosed endoscopically. The impact of GORD and its treatment on ILD features (including severity and time to ILD development) and survival was evaluated. RESULTS: GORD was a common manifestation affecting 1539/1632 (94%) of SSc patients. GORD affected 450/469 (96%) of those with SSc-ILD cohort. In SSc-ILD, there was no relationship between the presence of GORD or its treatment and time to ILD development or ILD severity. However, GORD treatment was associated with improved survival in those with ILD (p = 0.002). Combination therapy with both a PPI and a H2RA was associated with a greater survival benefit than single agent therapy with PPI alone (HR 0.3 vs 0.5 p < 0.050 respectively). CONCLUSION: GORD is a common SSc disease manifestation. While the presence or treatment of GORD does not influence the development or severity of ILD, aggressive GORD treatment, in particular with a combination of PPI and H2RA, is associated with improved survival in those with SSc-ILD.

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OBJECTIVE: To review the current literature, and evaluate the psychometric properties of disease damage indices in rheumatic diseases. METHODS: A search of Medline, EMBASE, and Cochrane Library databases was performed to June 2018 to identify damage indices in all systemic rheumatic diseases. Articles were included in a systematic review if indices were composite (multi-organ) in nature and if adequate detail on methodology was described. Articles pertaining to the validation of these indices were also reviewed in order to assess the psychometric properties of the indices using the Outcome Measures in Rheumatology Arthritis Clinical Trials (OMERACT) filter as a guide. RESULTS: Of the 2659 articles retrieved through the search, we identified 7 damage indices in five diseases: idiopathic inflammatory myopathy, systemic lupus erythematosus, systemic vasculitis, SjÓ§gren's syndrome and antiphospholipid syndrome. A further 48 articles were identified pertaining to the validation of these damage indices. The methodological process for the development of these indices included expert consensus, item reduction and item weighting methods. The level of validation that these indices have achieved is variable, with only 2 damage indices fulfilling all criteria of the OMERACT filter. CONCLUSIONS: To date, there have been 7 composite disease damage indices created in a variety of rheumatic diseases, with the exception of systemic sclerosis (SSc). This review has informed methodology for the development of a disease damage index in SSc.

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AIMS: To study student's knowledge of the effects of sunlight on the eyes, as well as their sun protective behaviours. METHODS: In total, 640 [corrected] students aged 13-18 years were surveyed in South Australia, during August-September 2004, using a standardized previously used survey. Scores were calculated regarding knowledge about ultraviolet light, sunlight effects on eyes, as well as eye and body protection. Risk factor scores were produced for each student. The data were analysed by the analysis of variance (ANOVA), as well as the Cochran-Mantel-Haenszel methods. Results were compared to the same survey conducted in 1995 in Queensland Australia. RESULTS: This group demonstrated a moderate level of knowledge, similar to the 1995 survey. Students in the older age groups demonstrated significantly higher knowledge. The majority of students (74%) owned a pair of sunglasses; however, 44.5% almost never wore their glasses. The reported frequency of wearing sunglasses was significantly related to advertising, believing sunglasses protect the eyes, as well as personal, family, and peer attitudes towards wearing sunglasses. CONCLUSION: The results of our survey suggest no significant change in knowledge and behaviours of students, compared to the 1995 survey. We feel it is imperative that adolescents be made more aware of the damaging effects of sunlight and the benefits of eye protection. Health promotion campaigns should target the youth and consider that as a group, they are significantly influenced by the media, peers, and family attitudes.

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