ABSTRACT
BACKGROUND: Screening for pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc) is now standard care in this disease. The existing Australian Scleroderma Interest Group algorithm (ASIGSTANDARD ) is based on transthoracic echocardiography (TTE) and pulmonary function tests (PFT). Recently, ASIG has derived and validated a new screening algorithm (ASIGPROPOSED ) that incorporates N-terminal pro-B-type natriuretic peptide level together with PFT in order to decrease reliance on TTE, which has some limitations. Right heart catheterisation (RHC) remains the gold standard for the diagnosis of PAH in patients who screen 'positive'. AIM: To compare the cost of PAH screening in SSc with ASIGSTANDARD and ASIGPROPOSED algorithms. METHODS: We applied both ASIGSTANDARD and ASIGPROPOSED algorithms to 643 screen-naïve SSc patients from the Australian Scleroderma Cohort Study (ASCS), assuming a PAH prevalence of 10%. We compared the costs of screening, the number of TTE required and both the total number of RHC required and the number of RHC needed to diagnose one case of PAH, and costs, according to each algorithm. We then extrapolated the costs to the estimated total Australian SSc population. RESULTS: In screen-naïve patients from the ASCS, ASIGPROPOSED resulted in 64% fewer TTE and 10% fewer RHC compared with ASIGSTANDARD , with $1936 (15%) saved for each case of PAH diagnosed. When the costs were extrapolated to the entire Australian SSc population, there was an estimated screening cost saving of $946 000 per annum with ASIGPROPOSED , with a cost saving of $851 400 in each subsequent year of screening. CONCLUSIONS: ASIGPROPOSED substantially reduces the number of TTE and RHC required and results in substantial cost savings in SSc-PAH screening compared with ASIGSTANDARD .
Subject(s)
Algorithms , Cost Savings/methods , Hypertension, Pulmonary/economics , Mass Screening/economics , Scleroderma, Systemic/economics , Aged , Cohort Studies , Echocardiography/economics , Echocardiography/methods , Female , Humans , Hypertension, Pulmonary/diagnosis , Male , Mass Screening/methods , Middle Aged , Prospective Studies , Respiratory Function Tests/economics , Respiratory Function Tests/methods , Scleroderma, Systemic/diagnosisABSTRACT
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.
Subject(s)
Gastroesophageal Reflux , Histamine H2 Antagonists , Lung Diseases, Interstitial , Proton Pump Inhibitors , Scleroderma, Systemic , Humans , Gastroesophageal Reflux/drug therapy , Gastroesophageal Reflux/complications , Lung Diseases, Interstitial/drug therapy , Female , Male , Middle Aged , Scleroderma, Systemic/complications , Scleroderma, Systemic/drug therapy , Proton Pump Inhibitors/therapeutic use , Aged , Histamine H2 Antagonists/therapeutic use , Adult , Cohort Studies , Treatment Outcome , Australia/epidemiologyABSTRACT
Epidemiological studies reporting demographic, clinical and serological factors predictive of various outcomes in systemic sclerosis (SSc) range from the prediction of mortality to the development and progression of disease manifestations. However, predicting the disease trajectory in the individual patient is a challenging but important step towards a stratified approach to disease management. Recent technological advances provide the opportunity for new subgroupings of disease based on risk stratification, through the systematic analysis of high-dimensional clinical data combined with genes, their transcription products and their corresponding translated proteins. In addition, these variables offer a rich vein of research to identify non-invasive biomarkers for predicting organ involvement and to assess disease activity and response to therapy. Selection of patients with a clinical phenotype or molecular signature relevant to the therapy under study combined with recent efforts to standardise outcome measures, show promise for improving clinical trial design and the identification of effective targeted therapies.