Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: results from a real-life screening programme

Pulmonary arterial hypertension (PAH) is the leading cause of death in systemic sclerosis (SSc). Annual screening with echocardiogram (ECHO) is recommended. We present the methodological aspects of a PAH screening programme in a large Australian SSc cohort, the epidemiology of SSc-PAH in this cohort, and an evaluation of factors influencing physician adherence to PAH screening guidelines.Patient characteristics and results of PAH screening were determined in all patients enrolled in a SSc longitudinal cohort study. Adherence to PAH screening guidelines was assessed by a survey of Australian rheumatologists. Summary statistics, chi-square tests, univariate and multivariable logistic regression were used to determine the associations of risk factors with PAH.Among 1636 patients with SSc, 194 (11.9%) had PAH proven by right-heart catheter. Of these, 160 were detected by screening. The annual incidence of PAH was 1.4%. Patients with PAH diagnosed on subsequent screens, compared with patients in whom PAH was diagnosed on first screen, were more likely to have diffuse SSc (p = 0.03), be in a better World Health Organisation (WHO) Functional Class at PAH diagnosis (p = 0.01) and have less advanced PAH evidenced by higher mean six-minute walk distance (p = 0.03), lower mean pulmonary arterial pressure (p = 0.009), lower mean pulmonary vascular resistance (p = 0.006) and fewer non-trivial pericardial effusions (p = 0.03). Adherence to annual PAH screening using an ECHO-based algorithm was poor among Australian rheumatologists, with less than half screening their patients with SSc of more than ten years disease duration.PAH is a common complication of SSc. Physician adherence to PAH screening recommendations remains poor. Identifying modifiable barriers to screening may improve adherence and ultimately patient outcomes.Kathleen Morrisroe, Wendy Stevens, Joanne Sahhar, Candice Rabusa, Mandana Nikpour, Susanna Proudman and the Australian Scleroderma Interest Group (ASIG

Pragmatics of Yes/No Indirect-responses (YNIRs)

AbstractHow do people transmit information with “question-answer” structures? What happens when a speaker utters a meaningful question and the hearer understands it? The present paper focuses on YNIRs in terms of (a) a radical lack of consensus about their potential in production of messages in interpersonal communication; (b) the ways in which they are used to establish and maintain coherent conversation, and (c) to what extent commentary, and supplementary indirect responses can invoke goal (in) compatibility, and how this kind of conflict can prevent stagnation, stimulate interest, and finally contribute to “escalation” of mutual understanding. Although the functional horizons of general questions and the answers to them vary from context to context, the addressee can “control” his judgements and attitudes (apology, ignorance, consent, or refusal) by his deeper exposure to the situation, what, in the end, enables his affiliation with others. The other major concern of the paper is to specify the cases when the pragmatic interpretation of questionless responses is defined as unification of the semantic representation and the internal utterance context

Quantifying the direct public health care cost of systemic sclerosis

To quantify the direct healthcare cost of systemic sclerosis (SSc) and identify its determinants. Healthcare use was captured through data linkage, wherein clinical and medication data for SSc patients from the state of Victoria enrolled in the Australian Scleroderma Cohort Study were linked with the Victorian hospital admissions and emergency presentations data sets, and the Medicare Benefits Schedule which contains all government subsidized ambulatory care services, for the period 2011-2015. Medication cost was determined from the Pharmaceutical Benefits Scheme. Costs were extrapolated to all Australian SSc patients based on SSc prevalence of 21.1 per 100,000 and an Australian population of 24,304,682 in 2015. Determinants of healthcare cost were estimated using logistic regression. Total healthcare utilization cost to the Australian government extrapolated to all Australian SSc patients from 2011 to 2015 was Australian Dollar (AUD)$297,663,404.77, which is an average annual cost of AUD$59,532,680.95 (US Dollar [USD]$43,816,040.08) and annual cost per patient of AUD$11,607.07 (USD$8,542.80). Hospital costs, including inpatient hospitalization and emergency department presentations, accounted for the majority of these costs (44.4% of total), followed by medication cost (31.2%) and ambulatory care cost (24.4%). Pulmonary arterial hypertension (PAH) and gastrointestinal (GIT) involvement were the major determinants of healthcare cost (OR 2.3 and 1.8, P = .01 for hospitalizations; OR 2.8 and 2.0, P = .01 for ambulatory care; OR 7.8 and 1.6, P < .001 and P = .03 for medication cost, respectively). SSc is associated with substantial healthcare utilization and direct economic burden. The most costly aspects of SSc are PAH and GIT involvement.Kathleen Morrisroe, Wendy Stevens, Joanne Sahhar, Gene-Siew Ngian, Candice Rabusa, Nava Ferdowsi, Catherine Hill, Susanna Proudman, Mandana Nikpou

Survival and quality of life in incident systemic sclerosis-related pulmonary arterial hypertension

Background: Pulmonary arterial hypertension (PAH) is a leading cause of mortality in systemic sclerosis (SSc). We sought to determine survival, predictors of mortality, and health-related quality of life (HRQoL) related to PAH in a large SSc cohort with PAH. Methods: We studied consecutive SSc patients with newly diagnosed (incident) World Health Organization (WHO) Group 1 PAH enrolled in a prospective cohort between 2009 and 2015. Survival methods were used to determine age and sex-adjusted standardised mortality ratio (SMR) and years of life lost (YLL), and to identify predictors of mortality. HRQoL was measured using the Short form 36 (SF-36) instrument. Results: Among 132 SSc-PAH patients (112 female (85%); mean age 62 ± 11 years), 60 (45.5%) died, with a median (±IQR) survival time from PAH diagnosis of 4.0 (2.2-6.2) years. Median (±IQR) follow up from study enrolment was 3.8 (1.6-5.8) years. The SMR for patients with SSc-PAH was 5.8 (95% CI 4.3-7.8), with YLL of 15.2 years (95% CI 12.3-18.1). Combination PAH therapy had a survival advantage (p < 0.001) compared with monotherapy, as did anticoagulation compared with no anticoagulation (p < 0.003). Furthermore, combination PAH therapy together with anticoagulation had a survival benefit compared with monotherapy with or without anticoagulation and combination therapy without anticoagulation (hazard ratio 0.28, 95% CI 0.1-0.7). Older age at PAH diagnosis (p = 0.03), mild co-existent interstitial lung disease (ILD) (p = 0.01), worse WHO functional class (p = 0.03) and higher mean pulmonary arterial pressure at PAH diagnosis (p = 0.001), and digital ulcers (p = 0.01) were independent predictors of mortality. Conclusions: Despite the significant benefits conferred by advanced PAH therapies suggested in this study, the median survival in SSc PAH remains short at only 4 years.Kathleen Morrisroe, Wendy Stevens, Molla Huq, David Prior, Jo Sahhar, Gene-Siew Ngian, David Celermajer, Jane Zochling, Susanna Proudman, Mandana NikpourEmail author and the Australian Scleroderma Interest Group (ASIG

The economic burden of systemic sclerosis related pulmonary arterial hypertension in Australia

BACKGROUND: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$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.Kathleen Morrisroe, Wendy Stevens, Joanne Sahhar, Gene-Siew Ngian, Nava Ferdowsi, Dylan Hansen, Shreeya Patel, Catherine L. Hill, Janet Roddy, Jennifer Walker, Susanna Proudman and Mandana Nikpou

Clinical characteristics and survival of pulmonary arterial hypertension with or without interstitial lung disease in systemic sclerosis.

Objectives: 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 (SSconly). 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-regres‑ sion modelling. Results: Of 1561 participants, 7% fulflled criteria for PAH-only, 24% ILD-only, 7% PAH-ILD and 62% SSc-only. People with PAH-ILD were more frequently male, with difuse skin involvement, higher infammatory markers, older age of SSc onset and higher frequency of extensive ILD than the cohort overall (p<0.001). People of Asian race more fre‑ quently developed PAH-ILD (p<0.001). People with PAH-ILD or PAH-only had worse WHO functional class and 6-minwalk-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.Jessica L. Fairley, Dylan Hansen, Laura Ross, Susanna Proudman, Joanne Sahhar, Gene, Siew Ngian, Jennifer Walker, Lauren V. Host, Kathleen Morrisroe, Diane Apostolopoulous, Nava Ferdowsi, Michelle Wilson, Maryam Tabesh, Wendy Stevens, Mandana Nikpour, and Australian Scleroderma Interest Grou

A historical reflection on literacy, gender and opportunity: implications for the teaching of literacy in early childhood education

This paper presents a historical reflection on gender and literacy, with a view to informing the present teaching of literacy in early childhood. The relationship between gender, literacy and opportunity in the labour market is examined, given that despite girls’ achievement in literacy, in comparison with boys’, women continue to earn substantially less than men. In order to understand this relationship, this paper reflects on literacy as a socio-historical construct as well as examining the ways in which the past is constitutive in forming enduring notions of gender that penetrate all elements of society, including the literacy classroom. This critical analysis of what is learned about and through the medium of literacy in the early childhood classroom has major implications for the teaching of literacy today. It is argued that in order to address this issue, early childhood educators need to value and nurture children’s digital literacies as well as create learning environments that allow all children genuine opportunities to question, challenge and explore dominant discourses that are embedded in text

Musculoskeletal manifestations of systemic sclerosis

Abstract not availableKathleen B. Morrisroe, Mandana Nikpour, Susanna M. Proudma