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OBJECTIVE: 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.

OBJECTIVE: Regular clinical assessment for complications of systemic sclerosis (SSc) such as pulmonary arterial hypertension (PAH) is essential for early institution of therapy and improved outcomes. The objective of this study was to determine the impact of COVID-19 pandemic-related restrictions on health care access of patients with SSc, including screening for PAH. METHODS: South Australian and Victorian patients enrolled in the Australian Scleroderma Cohort Study were surveyed about their perceptions of the impact of the pandemic on mental well-being, access to medications, investigations, and management of SSc. Frequency of annual rheumatology assessments, pulmonary function tests (PFT), and transthoracic echocardiography (TTE) to screen for PAH were compared with rates from before the pandemic. RESULTS: A total of 312 of 810 patients with SSc responded (38.5% response); 273 were female (87.5%), the median age was 64.7 years, 77.2% had limited disease, the median illness duration was 15.6 years, 15.7% were immunosuppressed, 32.1% had interstitial lung disease, and 6.4% had PAH. A total of 65.7% of consultations were by telehealth, of which 81.2% were by telephone. Compared with respondents in South Australia (n = 109), Victorian respondents (n = 203) experiencing prolonged lockdown, reported reduced access to their rheumatologist (49.3% vs 27.9%; P = 0.004), greater use of consultation by video (17.3% vs 2.1%; P = 0.008), greater health care disruption (49.0% vs 23.2%; P < 0.001), and worse mental health (P = 0.002). Respondents reported reduced access to PFT and TTE (31.7% and 22.5%, respectively). Annual visits, PFT, TTE, and new diagnoses of PAH were reduced in 2020 to 2022 compared with 2011 to 2019. CONCLUSION: The COVID-19 pandemic-related disruption to health care for patients with SSc was associated with worse mental health and reduced screening and diagnosis of PAH, which may impact long-term outcomes.

Background/Purpose: Metabolic syndrome (MetS) is a chronic pro-inflammatory and pro-thrombotic state associated with increased atherosclerosis, cardiovascular events and type 2 diabetes. It is diagnosed in the presence of at least 3 of 5 metabolic risk factors (obesity, hypertension, hypertriglyceridemia, low high-density lipoproteins (HDL) and insulin resistance [1]). The pathophysiology of MetS remains incompletely understood, but may share mechanisms with inflammatory states such as SLE. Visceral adiposity is known as a source of pro-inflammatory cytokines by adipocytes, the so-called adipokines. The contribution of glucocorticoids to the development of MetS in SLE is not clear, even though their use is associated with cardiovascular comorbidities [2]. We aim to characterize the prevalence and associations of MetS, including serum levels of adipokines, in a multi-ethnic cohort of patients with SLE. Method(s): Using a standardised protocol, baseline demographics, per visit disease activity (SLEDAI2K) and treatment data, and annual recording of organ damage accrual (SLICC damage index (SDI)) were prospectively captured on SLE patients from a single tertiary centre. The presence of MetS,defined using the updated joint consensus criteria [1], was assessed retrospectively at the last recorded visit. Serum levels of adipokines (resistin, lipocalin-2, TNF, MCP-1) and insulin were measured by Quantibody. Result(s): 116 patients (median (IQR) age at enrolment 39.5 (31.4-51.1) years, median disease duration 6.1 (1.4-12) years) were followed for a median of 6.7 (4.1-8.1) years. 80% of patients were exposed to glucocorticoids (median time-adjusted mean dose 3.7 (0.5-7.0) mg/day and 50% accrued organ damage during follow-up. The prevalence of MetS in this cohort was 29%. The prevalence of the components of MetS included: hypertension (59%), low-HDL (51%), hypertriglyceridemia (32%), obesity (16%) and hyperglycaemia (22%). In univariable analysis, MetS was associated with base

Objectives To characterise the prevalence and associations of metabolic syndrome (MetS) in a multiethnic cohort of patients with SLE. Methods Using a standardised protocol, baseline demographics, per visit disease activity (Systemic Lupus Erythematosus Disease Activity Index-2K) and treatment data, and annual recording of organ damage accrual (Systemic Lupus International Collaborating Clinics/American College of Rheumatology (SLICC-ACR) Damage Index) were captured on patients with SLE from a single tertiary centre. The presence of MetS, defined using modified updated joint consensus criteria, was assessed at the final visit from patient records. Serum concentrations of adipocytokines were measured by Quantibody. Results 116 patients, with median (Q1, Q3) age at enrolment of 39.5 (31.4-51.1) years and disease duration of 6.1 (1.4-12) years, were followed for a median of 6.7 (4.1-8.1) years. The prevalence of MetS was 29% (34/116), while the prevalence of MetS components varied: hypertension (59%), low high-density lipoproteins (HDLs) (51%), hypertriglyceridaemia (32%), obesity (16%) and hyperglycaemia (22%). In univariable analysis, MetS was associated with baseline organ damage (OR 4.34; 95% CI 1.80 to 10.48; p<0.01) and organ damage accrual (OR 2.34; 95% CI 1.02 to 5.36; p=0.04) but not with disease activity. In multivariable analysis, baseline organ damage remained significantly associated with MetS (adjusted OR 3.36; 95% CI 1.32 to 8.59; p=0.01). Glucocorticoid use was not associated with MetS or any of its five components. High serum concentrations of resistin were significantly negatively associated with MetS (OR 0.17; 95% CI 0.04 to 0.70; p=0.014). Conclusion MetS was common in a multiethnic cohort of patients with SLE, with the most frequent components being hypertension and low HDL. An independent association was found between MetS and organ damage but not glucocorticoid exposure or disease activity.Copyright ©

Aim. DEXA scans allow for measurement of body composition (fat mass, lean muscle mass, bone mineral content(BMC)) and also specify distribution of each component. To determine body composition changes in lupus patients that gained/lost significant weight over time, and determine effect of glucocorticoids. Method. Patients of the Monash Lupus Cohort 2007-2015 that were enrolled for at least 12 months and had at least two DEXA with minimum 1 year separation. Body Composition data was extracted from radiological files. Significant weight change was defined as >7% change from baseline. Result. 68 patients were included in this study. Mean time between scans was 3.4 years. 16% gained significant weight and 15% lost significant weight during the study. Comparing the three groups (weight gain; weight loss; minimal change) there were no significant differences in body composition at baseline. Overall, there was very little difference in change in body composition and distribution. The weight loss group had lower total fat mass and total lean muscle mass (-7909g and-2777g respectively) compared to baseline. There was a reduction in both android and gynoid fat percentages compared with baseline (-9.6%,-5.31% respectively). Significant weight loss was positively associated with time-adjusted-mean-prednisolone >4.42mg/day (OR 4.66, p=0.02) and negatively associated with Asian ethnicity (OR 0.07, p=0.01) in multivariate analysis. The weight gain group had increases in total fat mass with minimal change in other components. There was an increase in both android and gynoid fat percentage compared with baseline (10.3% and 6.4%). Significant weight gain was negatively associated with Asian ethnicity (OR 0.23, p=0.02) and positively associated with higher baseline ESR (OR6.28, p<0.01) and neutrophilia (OR 6.58, p<0.01) in multivariate analysis. Conclusions. Weight loss occurred in both fat and muscle components and was associated with higher glucocorticoid exposure. Asian ethnicity w

Background: The Metabolic Syndrome (MetS) is a disorder of energy utilisation and storage, associated with an increased risk of cardiovascular (CV) disease. MetS may contribute to the increased CV disease in SLE, but the prevalence, cause, and impact of MetS in SLE is poorly understood, as are the effects of glucocorticoid (GC) exposure. Objective(s): To characterise the prevalence of the features of MetS in a wellcharacterised cohort of SLE patients, and determine the effect of GC use on these parameters. Method(s): SLE patients studied as part of a single centre prospective longitudinal cohort. Disease activity (SLEDAI-2K), treatment and laboratory details were recorded at each visit. Other investigation results were collected from institution databases. MetS defined as>=3 criteria1: BMI>30kg/m2; triglycerides>1.7mmol/L; HDL-cholesterol <1.3mmol/L; blood pressure >130/85mmHg or treatment for hypertension; fasting glucose >5.6mmol/L or treatment for hyperglycaemia. Continuous variables were described as median (IQR), and compared using Kruskal-Wallis tests. Categorical variables were described as frequency and compared using Chi-squared tests. Result(s): 289 patients were included (87% female; 51% Caucasian, 29% Asian), and median age at enrolment of 37.7y. Median follow-up was 3.43y (med 15 visits). Time adjusted-mean SLEDAI (AMS) over the study period was 3.67. 81% (211) patients received GC (time-adjusted mean 4.25mg prednisolone/d) and AMS was significantly higher in GC-exposed patients (4.19 vs 1.97 [EM1], p<0.01). MetS criteria were met by 49 (17%) of patients (Table 1). Hypertriglyceridaemia and hypertension were significantly more frequent in GC-treated patients, but the prevalence of obesity and other MetS domains, or MetS overall, were not. There were significantly more patients with MetS score =0 in the GC-exposed subset (43/78 vs 76/211 p<0.01). The prevalence of obesity of 17% is lower than in the general population. There was no significant change in

Aim. The Metabolic Syndrome is a disorder of energy utilisation and storage, associated with an increased risk of cardiovascular disease. Metabolic Syndrome may contribute to the increased CV disease in SLE. To characterise the prevalence and features of Metabolic Syndrome in a cohort of SLE patients, and determine predictors of development of the Metabolic Syndrome. Method. SLE patients (ACR>4) were studied from 2007-2015. Baseline demographic data, disease activity, and treatment data were captured as per protocol. Components of Metabolic Syndrome were attained from patient records. Metabolic Syndrome defined as >3 criteria: BMI >30kg/m2; triglycerides>1.7mmol/L; HDL-cholesterol <1.3mmol/L; BP >130/85mmHg or treatment; fasting glucose >5.6mmol/L or treatment. Result. There were 286 patients in the study, with median age at diag-nosis 29.5years (IQR 22.6-42.8). Median baseline SLEDAI was 4 (2-6) and the time-adjusted disease activity (AMS) was 3.7 (2-5.3). 209/286 (73%) of patients were exposed to glucocorticoids. The prevalence of components of the Metabolic Syndrome were hypertension 57%, obesity 20%, hyperglycemia 23%, low HDL 50%, hypertriglyceridemia 35%. Multivariate analysis revealed that hypertension was associated with male gender (OR 3.58, p=0.02), older age at diagnosis (OR 2.47, p=0.02), obesity at baseline (OR 2.76, p=0.01) and MMF exposure (OR 3.13, p<0.01). Asian ethnicity was found to be significantly nega-tively associated with obesity in multivariate analysis (OR 0.34, p=0.03). Obesity was more prevalence in patient group not exposed to glucocorticoid (14% vs 32%, p=0.04). No association was found between glucocorticoid exposure and hyperglycemia. Hydroxychloroquine was positively associated with low HDL (OR 6.66, p=0.02). Metabolic Syndrome was diagnosed in 37% of cohort. Patients with Metabolic Syndrome had older age at diagnosis (45 vs 38years, p<0.01), more damage at baseline (SDI=1 vs 0, p<0.01), and no association with glucocorticoid exposur

Objectives: To determine factors associated with damage accrual in a prospective cohort of patients with SLE. Method(s): Patients with SLE who attended the Lupus Clinic at Monash Health, Australia, between 2007 and 2013 were studied. Clinical variables included disease activity (Systemic Lupus Erythematosus Disease Activity Index-2K, SLEDAI-2K), time-adjusted mean SLEDAI, cumulative glucocorticoid dose and organ damage (Systemic Lupus International Collaborating Clinics Damage Index (SDI)). Multivariate logistic regression analyses were performed to identify factors associated with damage accrual. Result(s): A total of 162 patients were observed over a median (IQR) 3.6 (2.0-4.7) years. Seventy-five per cent (n=121) of patients received glucocorticoids. Damage accrual was significantly more frequent in glucocorticoid-exposed patients (42% vs 15%, p<0.01). Higher glucocorticoid exposure was independently associated with overall damage accrual after controlling for factors including ethnicity and disease activity and was significant at time-adjusted mean doses above 4.42 mg prednisolone/day; the OR of damage accrual in patients in the highest quartile of cumulative glucocorticoid exposure was over 10. Glucocorticoid exposure was independently associated with damage accrual in glucocorticoid-related and nonglucocorticoid related domains of the SDI. Conclusion(s): Glucocorticoid use is independently associated with the accrual of damage in SLE, including in non-glucocorticoid related domains.

The treatment of SLE remains complex, and management is constrained by a lack of safe, effective, targeted therapies. Physicians, also, are constrained by a lack of evidence-based approaches with existing agents, including glucocorticoids, utilized in the majority of patients. While Cushingoid side effects of glucocorticoids are widely recognized, emerging literature now suggests that glucocorticoid use actually contributes to harmful outcomes in SLE, over and above these effects. These studies provide a compelling case for a re-evaluation of the long-term use of glucocorticoids in SLE, focusing on minimizing glucocorticoid exposure as part of the strategy to improve long-term outcomes. In this article, we review the evidence for the harmful effects of glucocorticoids in SLE, and propose therapeutic options that reduce reliance on glucocorticoids. We propose that it is time for the lupus community to have a louder conversation about glucocorticoid use, and for any residual complacency about their risk-benefit ratio to be banished.Copyright © The Author 2016. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For Permissions, please email: journals.permissions@oup.com.

Aim: Glucocorticoids remain the mainstay of systemic lupus erythematosus (SLE) therapy in both acute and chronic settings, despite their numerous well-known adverse effects. We aimed to quantify clinical outcomes potentially related to glucocorticoid adverse effects in a cohort of patients with SLE. Method(s): SLE patients (ACR >= 4) were studied longitudinally in a single centre from 2007-2013. Disease activity (SLEDAI-2k) and treatment details were recorded at each visit. Relevant investigation results were retrospectively collected from institution databases. Outcomes analysed included obesity, osteoporosis/paenia hypertension, diabetes mellitus, dyslipidemia and coronary artery disease (CAD). Result(s): 192 patients were observed for a mean 3.72 +/- 1.98 years. 148 (77%) received glucocorticoids (mean +/- SD 7.93 +/- 6.15 mg/d). The prevalence of outcomes was: obesity (n = 32/192, 17%), osteoporosis (n = 29/ 192, 15%), hypertension (n = 19/192, 10%), diabetes mellitus (n = 7/192, 4%), dyslipidemia (n = 10/192, 10%) and CAD (n = 4/192, 2%). A highly significant association between glucocorticoid use and reduced bone density (osteoporosis/penia) (47/148 vs 5/44, p = 0.007), but not osteoporosis (24/148 vs 5/44, p = 0.63). There was no significant association between hypertension, diabetes, dyslipidemia or CAD with the use of glucocorticoids. Unexpectedly, final BMI was significantly less in glucocorticoid-exposed patients (24.11 +/- 0.41 vs 28.50 +/- 1.28, p < 0.0001). Glucocorticoidexposed patients had lower baseline BMI (23.74 +/- 0.39 vs 28.24 +/- 1.41, p < 0.0001), but no significant difference in weight change. Accordingly there was no correlation between cumulative glucocorticoid exposure and change in BMI. Although increased disease activity was observed in the glucocorticoid-exposed group (p < 0.0001) change in BMI did not correlate with time-adjusted mean SLEDAI. Conclusion(s): As expected, a significant association between glucocorticoid exposure and red

Aim: Systemic lupus erythematosus (SLE) is a chronic autoimmune disease in which inflammation causes irreversible organ damage. The majority of SLE patients receive treatment with glucocorticoids, which potentially contributes independently to organ damage. We aimed to determine whether glucocorticoid use is associated with organ damage in SLE. Method(s): SLE patients (ACR >= 4) were studied from 2007-2012. Disease activity (SLEDAI-2k) and treatment details were recorded at each visit, and organ damage measured annually (SLICC-SDI). Result(s): 184 patients were included; 138 received glucocorticoids during the study period (mean 8.4 +/- 0.5 mg/day). Patients exposed to glucocorticoids had significantly more severe disease, as indicated by significantly higher time-adjusted mean SLEDAI (AMS) during the period of observation (5.0 +/- 0.3 vs. 2.2 +/- 0.3, P < 0.0001) and a significantly higher mean number of clinic visits (15.5 +/- 0.9 vs. 8.6 +/- 0.8, P < 0.0001). Final SDI was significantly worse in glucocorticoid-exposed patients (P = 0.02), as were change in SDI from baseline (P = 0.01) and rate of change in SDI (P = 0.04). Patients in the 4th quartile of glucocorticoid exposure had significantly higher final SDI (P = 0.0006) and change in SDI (P = 0.0005) compared to the 1st quartile. We calculated an adjusted SDI score (PNL-SDI) including only damage events (osteoporosis, diabetes, cataracts, avascular necrosis) directly induced by glucocorticoids, as well as a score additionally including cardiovascular damage events (PNL+CVSDI). In glucocorticoid-exposed but not non-exposed patients, significant increases in PNL-SDI (P = 0.0005) and PNL+CV-SDI (P < 0.0001) were observed over the study period, which were again more significant in patients in the 4th quartile of glucocorticoid exposure compared to the 1st quartile. Conclusion(s): Not with standing the direct effects of disease activity on outcomes, and the association between high disease activity and glucocortic

Aim: In systemic lupus erythematosus (SLE), organ damage occurs in response to autoimmune-induced inflammation but also glucocorticoid therapy. Inflammatory activity and damage are measured with validated instruments, but the glucocorticoid-induced organ damage is not routinely quantified. We evaluated whether a modified damage index could be utilised to assess glucocorticoid-induced damage in SLE. Method(s): SLE patients (ACR >= 4) were studied 2007-2012. Disease activity (SLEDAI-2k) and treatment details were recorded at each visit, and organ damage measured annually (SLICC-SDI). Four domains of the SDI that relate directly to glucocorticoid use (osteoporosis, avascular necrosis, diabetes mellitus and cataracts) were combined to form the glucocorticoidassociated SDI (GC-SDI). Correspondingly, all other domains of the SDI comprised the non-GC-SDI. Result(s): 184 patients were included; 138 received glucocorticoids during the study period (mean 8.4 +/- 0.5 mg/d). Baseline SDI was not significantly different in the subsets exposed to glucocorticoids, however final SDI was significantly worse in glucocorticoid-exposed patients (p = 0.02). There was a strong correlation between time-adjusted mean SLEDAI (AMS) and cumulative prednisolone usage (p < 0.0001). Total SDI was significantly correlated with AMS, r = 0.258, p = 0.0004) and with cumulative prednisolone dose (r = 0.269, p = 0.0002), indicating that damage accrual is associated with both disease activity and exposure to prednisolone. GC-SDI was not significantly associated with disease activity (AMS) in either all patients (r = 0.075, p = 0.31) or in glucocorticoid exposed patients (r = 0.038, p = 0.65). In glucocorticoid users, cumulative prednisolone exposure was correlated with change in GC-SDI (r = 0.214, p = 0.012) and with final GC-SDI (r = 0.16, p = 0.063). The GC-SDI was also found to be positively correlated with greater clinic visits (p = 0.05), and duration of SLE (p = 0.0009). The non-GC-SDI was signif

Aim: We aimed to evaluate the characteristics of patients presenting to Emergency Departments (ED) with low back pain (LBP), and whether ED physiotherapy review influences hospital admission. Method(s): We performed a retrospective review of all presentations to ED coded as LBP between 1/1-31/12/2012, at two hospitals (Monash Medical Centre (MMC), Dandenong Hospital (DH)) within the same health network. DH but not MMC ED has a policy regarding routine LBP referral to physiotherapy. Information regarding patient characteristics, physiotherapy reviews, and details of admission were collected utilising the e-audit function of the Symphony ED system. Result(s): There were 2248 presentations to ED with LBP and 389 (17%) resulted in admission. Admissions were more common for weekend (OR 1.43 (1.1-1.9), p = 0.0072) and ambulance presentations (OR 3.3 (2.6- 4.1, p < 0.0001). Admitted patients were younger (46 +/- 0.4 vs. 61 +/- 1, P < 0.0001) and compensible status was associated with reduced admissions (OR 0.53 (0.34-0.84, p = 0.0086). An increased frequency of physiotherapy review at DH (32%) compared with MMC (19%) (OR 2.0 (1.7-2.4), P < 0.0001) was accompanied by significantly lower frequency of admission (11% vs. 24%, OR 0.39 (0.32- 0.50), p < 0.0001). There was a strong association between physiotherapy review and discharge from ED at DH (OR 0.667, 95% CI 0.46-0.97, p = 0.0355). There were no significant site differences in age, sex, compensability status, type of ambulance use, time of day, day of week, or use of imaging. Conclusion(s): Low back pain is a frequent presentation to ED that frequently results in admission. The most prominent factor differentiating admission rates at two hospitals within same health network was the rate of physiotherapy review. This is strong evidence for the importance of routine physiotherapy review in the management of patients presenting with LBP to ED.

OBJECTIVES: To determine factors associated with damage accrual in a prospective cohort of patients with SLE. METHODS: Patients with SLE who attended the Lupus Clinic at Monash Health, Australia, between 2007 and 2013 were studied. Clinical variables included disease activity (Systemic Lupus Erythematosus Disease Activity Index-2K, SLEDAI-2K), time-adjusted mean SLEDAI, cumulative glucocorticoid dose and organ damage (Systemic Lupus International Collaborating Clinics Damage Index (SDI)). Multivariate logistic regression analyses were performed to identify factors associated with damage accrual. RESULTS: A total of 162 patients were observed over a median (IQR) 3.6 (2.0-4.7) years. Seventy-five per cent (n=121) of patients received glucocorticoids. Damage accrual was significantly more frequent in glucocorticoid-exposed patients (42% vs 15%, p<0.01). Higher glucocorticoid exposure was independently associated with overall damage accrual after controlling for factors including ethnicity and disease activity and was significant at time-adjusted mean doses above 4.42 mg prednisolone/day; the OR of damage accrual in patients in the highest quartile of cumulative glucocorticoid exposure was over 10. Glucocorticoid exposure was independently associated with damage accrual in glucocorticoid-related and non-glucocorticoid related domains of the SDI. CONCLUSIONS: Glucocorticoid use is independently associated with the accrual of damage in SLE, including in non-glucocorticoid related domains.

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterised by multi-system manifestations. It is regarded as the prototypal connective tissue disease, where the key pathogenesis relates to a dysfunctional immune system that results in over-production of various autoantibodies. Most of its pathology is mediated by either direct or indirect effects of these autoantibodies, as well as other components of the innate and adaptive immune systems.