The economic burden of systemic sclerosis-A systematic review.

AIM: Systemic sclerosis (SSc) is a rare, chronic autoimmune disease associated with a substantial economic burden. This study aimed to assess the costs associated with SSc and to identify major cost drivers. METHODS: A systematic search was conducted in MEDLINE and Embase to identify relevant studies. Two independent reviewers evaluated studies based on inclusion/exclusion criteria and performed data extraction. Costs were converted into 2017 US dollars by purchasing power parity. The review was conducted following the Preferred Reporting Items for Systematic Review and Meta-analysis (PRISMA) guideline. RESULTS: The original literature search identified 113 potentially relevant citations, of which 10 articles met all the inclusion/exclusion criteria and were included in the data extraction and analysis. The identified studies evaluated costs associated with SSc in 11 countries from North America, Europe, and Australia published between 2009 and 2018. Eight studies reported direct costs and seven studies reported indirect costs. Direct costs varied from $3356 (Hungary) to $27 032 (Germany) with hospitalization and medication being two of the biggest components of direct medical costs in most studies. The indirect costs for lost productivity varied from $2433 (Italy) to $20 663 (UK), accounting for a significant portion of the total economic burden. CONCLUSIONS: Large variations existed in annual costs of SSc, but all studies found that SSc imposed a substantial economic burden on patients and their families. The identified studies were mostly from high-income countries and there is still a knowledge gap regarding the cost of SSc in other parts of the world.

Productivity Loss in Patients With Chronic Diseases: A Pooled Economic Analysis of Hungarian Cost-of-Illness Studies.

OBJECTIVES: To assess productivity loss (PL) variations across a set of chronic diseases and analyze significant PL drivers (demographics, health status, healthcare resource use) in Hungary. METHODS: Data from 11 cost-of-illness studies (psoriasis, dementia, systemic sclerosis, multiple sclerosis, benign prostatic hyperplasia, Parkinson's disease, psoriatic arthritis, rheumatoid arthritis, schizophrenia, epilepsy, and diabetes) were pooled, and patient-level data were analyzed. A weighted multiple linear regression analysis was run to identify significant PL indicators. All costs were adjusted to 2018 euro rates and PL was further presented as a proportion of gross domestic product/capita, facilitating results comparability and transferability. RESULTS: The dataset comprised 1888 patients from 11 chronic diseases. The average indirect cost/(gross domestic product/capita) ratio was highest in schizophrenia (72.4%) and rheumatoid arthritis (71.3%) and lowest in benign prostatic hyperplasia (1.6%). Correlation results infer that a higher EuroQol 5-dimension 3-level index score was significantly associated with lower PL. The number of hospital admissions was the main contributor toward increasing PL among resource use indicators. Age and sex showed inconsistent and insignificant correlations with PL. In regression analysis, a better EuroQol 5-dimension 3-level index score and higher education were consistently associated with decreasing PL in all models. CONCLUSIONS: This article will enable health decision makers to understand the importance of adopting a societal perspective for chronic disease reimbursement decisions. The correlation between PL and health status supports that timely started effective treatments may prevent patients from losing their workability.

Healthcare Resource Utilization Among Patients in England with Systemic Sclerosis-Associated Interstitial Lung Disease: A Retrospective Database Analysis.

INTRODUCTION: Systemic sclerosis-associated interstitial lung disease (SSc-ILD) places a substantial burden on patients and healthcare systems. The objectives of this study were to describe clinical characteristics and assess healthcare resource utilization and costs of patients with SSc-ILD in England, compared with patients with non-pulmonary organ involvement related to SSc (SSc-OOI). METHODS: This population-based retrospective study used data from the Clinical Practice Research Datalink linked to Hospital Episode Statistics. Data were extracted from medical records dated January 1, 2005 to March 31, 2016. Patients with SSc were identified and placed in subgroups based on organ involvement: SSc-ILD, SSc-OOI, and both (SSc-ILD-OOI). Patients with SSc-ILD-OOI were included in both the SSc-ILD and SSc-OOI subgroups. All-cause healthcare costs, excluding medication costs, were calculated to 2016 British pounds sterling (£). RESULTS: This study included 675 patients with SSc: 174 (26%) had neither ILD nor other organ involvement (OOI); 127 (19%) had SSc-ILD; 477 (71%) had SSc-OOI; 103 (15%) had SSc-ILD-OOI. Age-weighted median [interquartile range (IQR)] annual healthcare costs per patient were: £1496 (£664-£2817) in SSc only; £6375 (£3451-£15,041) in SSc-ILD; £4084 (£1454-£10,105) in SSc-OOI; £6632 (£4023-£17,009) in SSc-ILD-OOI. In multivariate analysis, older age at diagnosis, diagnosis of anemia, and number of comorbid diseases were associated with higher yearly healthcare costs. CONCLUSION: The annual healthcare cost for patients with SSc-ILD is substantial, and higher than that of patients with SSc-OOI or SSc only. These results quantify the economic burden of SSc-ILD in a real-world setting, and highlight the need for treatment of this disease.

SSc is a rare disease that causes fibrosis, or thickening, of the skin. In some patients, SSc can also affect the lungs ('SSc-ILD') or other organs, e.g., the heart ('SSc-OOI'). Patients with SSc-ILD typically have high healthcare costs; however, it is not clear how costs for SSc-ILD compare with those for SSc-OOI. To investigate this, we evaluated the costs associated with SSc-ILD and compared them with those for SSc only or SSc-OOI. In this England-based study, the annual healthcare costs for patients with SSc-ILD were approximately 50% higher than for those without lung disease (SSc only) or SSc-OOI. These results highlight the importance of promptly diagnosing and treating patients with lung fibrosis complicating SSc.

The clinical and economic burden of systemic sclerosis related interstitial lung disease.

OBJECTIVE: 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 <10%, moderate 10-30%, severe >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<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<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<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<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<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.

Digital ulcers in systemic sclerosis: their epidemiology, clinical characteristics, and associated clinical and economic burden.

BACKGROUND: To determine the frequency and clinical characteristics of systemic sclerosis-related digital ulcers, and associated direct health care costs, quality of life, and survival. METHODS: Digital ulcers (DUs) were defined as an area with a visually discernible depth and a loss of continuity of epithelial coverage. DU severity was calculated based on the physician reported highest number of new DUs at clinical review (mild = 1-5 DUs, moderate 6-10 DUs, severe > 10 DUs). Healthcare use was captured through data linkage, wherein SSc clinical data captured prospectively in a dedicated clinical database were linked with health services databases to capture hospital admissions, emergency department (ED) presentations and ambulatory care (MBS) utilization and cost for the period 2008-2015. Healthcare cost determinants were estimated using logistic regression. RESULTS: Among 1085 SSc patients, 48.6% experienced a DU over a mean follow-up of 5.2 ± 2.5 years. Those who developed DUs were more likely to have diffuse disease subtype (34.9% vs 18.2%, p < 0.001), anti-Scl-70 antibody (18.9% vs 9.3%, p < 0.001), and a younger age at SSc onset (43.6 ± 13.9 vs 48.8 ± 14.0 years, p < 0.001) in addition to reduced health-related quality of life (HRQoL) measured by the SF-36 but without a significant impact on survival. SSc patients with a history of a DU utilized significantly more healthcare resources per annum than those without a DU, including hospitalizations, ED presentation, and ambulatory care services. Total healthcare services, excluding medications, were associated with an annual excess cost per DU patient of AUD$12,474 (8574-25,677), p < 0.001, driven by hospital admission and ED presentation costs. CONCLUSION: DUs place a large burden on the patient and healthcare system through reduced HRQoL and increased healthcare resource utilization and associated cost.

Cost savings with a novel algorithm for early detection of systemic sclerosis-related pulmonary arterial hypertension: alternative scenario analyses.

Pulmonary arterial hypertension is an important cause of death and disability in patients with systemic sclerosis (SSc). Yearly screening of all SSc patients with transthoracic echocardiography (TTE) is recommended in international guidelines and currently utilised by the Australian Scleroderma Interest Group (ASIGSTANDARD ). Owing to the limitations of TTE, the ASIG developed a new screening algorithm (ASIGPROPOSED ) utilising a serum biomarker, NT-proBNP, in place of TTE, which has been shown to be equally accurate as the current algorithm. The aim of this study was to compare the cost of these two algorithms using different scenarios. The new algorithm resulted in significant yearly cost savings of between AU$42 913.35 and AU$84 570 in screening and diagnosis of an Australian cohort which, if extrapolated to the Australian population, would result in a yearly cost saving of between AU$367 066 and AU$725 564. There was no scenario in which the proposed algorithm did not result in a cost saving.

Economic Burden of Illness Among Commercially Insured Patients with Systemic Sclerosis with Interstitial Lung Disease in the USA: A Claims Data Analysis.

INTRODUCTION: Interstitial lung disease (ILD) is a common manifestation of scleroderma/systemic sclerosis (SSc). However, the direct and indirect economic burdens of SSc-ILD remain unclear. This study assessed and compared healthcare resource utilization (HRU), direct healthcare costs, work loss, and indirect costs between patients with SSc-ILD and matched controls with neither SSc nor ILD in the USA. METHODS: Data were obtained from a large US commercial claims database (2005-2015). Patients (at least 18 years old) had at least one SSc diagnosis in the inpatient (IP) or emergency room (ER) setting or at least two SSc diagnoses in another setting, and at least one diagnosis of ILD in the IP or ER setting or at least two diagnoses of ILD in another setting. Controls with neither SSc nor ILD were matched 5:1 to patients with SSc-ILD. Comparisons were conducted using Wilcoxon signed-rank and McNemar's tests and adjusted odds ratios (ORs) and incidence rate ratios (IRRs). RESULTS: A total of 479 SSc-ILD patients and 2395 matched controls were included (52 SSc-ILD patients and 260 matched controls for work loss and indirect cost analyses). Patients with SSc-ILD had significantly higher HRU and costs, IP admissions (adjusted IRR = 5.6), IP hospitalization days (adjusted IRR = 12.0), ER visits (adjusted IRR = 2.8), OP visits (adjusted IRR = 3.1), and days of work loss (adjusted IRR = 4.5). The adjusted difference in annual direct healthcare costs was $28,632 (SSc-ILD, $33,195; controls, $4562) and that in indirect costs was $4735 (SSc-ILD, $5640; controls, $906) (all p < 0.0001). CONCLUSION: SSc-ILD patients had significantly higher HRU, work loss, and direct and indirect costs compared to matched controls with neither SSc nor ILD. FUNDING: Boehringer Ingelheim Pharmaceuticals, Inc.

Economic Burden among Commercially Insured Patients with Systemic Sclerosis in the United States.

OBJECTIVE: To quantify healthcare resource utilization (HRU), work loss, and annual direct and indirect healthcare costs among patients with systemic sclerosis (SSc) compared to matched controls in the United States. METHODS: Data were obtained from a large US commercial claims database. Patients were ≥ 18 years old at the index date (first SSc diagnosis) and had ≥ 1 SSc diagnosis in the inpatient (IP) or emergency room (ER) setting, or ≥ 2 SSc diagnoses on 2 different dates in the outpatient (OP) setting between January 1, 2005, and March 31, 2015; continuous enrollment was required during the followup period (12 months after the index date). Individuals with no SSc diagnoses were matched 1:1 to patients with SSc. Wilcoxon signed-rank and McNemar tests were used for comparisons and regressions with generalized estimating equations for adjusted OR (aOR) and incidence rate ratios (IRR) between 2 cohorts. RESULTS: There were 2192 pairs of patients with SSc and matched controls included (mean age 57.6 yrs; 84.3% female); of these, 233 were eligible for work loss/indirect cost analyses. Compared to matched controls, patients with SSc had significantly higher HRU and costs during the 1-year followup period, IP admissions (adjusted IRR = 2.4), IP hospitalization days (adjusted IRR = 3.1), ER visits (adjusted IRR = 2.0), OP visits (adjusted IRR = 2.3), and days of work loss (adjusted IRR = 2.6). The adjusted difference in annual direct and indirect costs was US$12,820 and $3103, respectively (all p < 0.0001). CONCLUSION: Patients with SSc had a high direct and indirect economic burden postdiagnosis.

Excess Productivity Costs of Systemic Lupus Erythematosus, Systemic Sclerosis, and Sjögren's Syndrome: A General Population-Based Study.

OBJECTIVE: To determine excess productivity losses and costs of systemic lupus erythematosus (SLE), systemic sclerosis (SSc), and Sjögren's syndrome (SS) at the population level. METHODS: Administrative databases from the province of British Columbia, Canada, were used to establish population-based cohorts of SLE, SSc, and SS, and matched comparison cohorts were selected from the general population. Random samples from these cohorts were surveyed about time absent from paid and unpaid work and working at reduced levels/efficiency (presenteeism), using validated labor questionnaires. We estimated excess productivity losses and costs of each diagnosis (over and above nonsystemic autoimmune rheumatic diseases [non-SARDs]), using 2-part models and work disability rates (not employed due to health). RESULTS: Surveys were completed by 167 SLE, 42 SSc, and 90 SS patients, and by 375 non-SARDs (comparison group) participants. Altogether, predicted excess hours of paid and unpaid work loss were 3.5, 3.2, and 3.4 hours per week for SLE, SSc, and SS patients, respectively. Excess costs were $86, $69, and $84 (calculated as 2015 Canadian dollars) per week, or $4,494, $3,582, and $4,357 per person annually, respectively. Costs for productivity losses from paid work stemmed mainly from presenteeism (SLE = 69% of costs, SSc = 67%, SS = 64%, and non-SARDs = 53%), not from absenteeism. However, many working-age patients were not employed at all, due to health (SLE = 36%, SSc = 32%, SS = 30%, and non-SARDs = 18%), and the majority of total productivity costs were from unpaid work loss (SLE = 73% of costs, SSc = 74%, SS = 60%, and non-SARDs = 47%). Adjusted excess costs from these unpaid production losses were $127, $100, and $82 per week, respectively, among SLE, SSc, and SS patients. CONCLUSION: In this population-based sample of prevalent SLE, SSc, and SS, lost productivity costs were substantial, mainly from presenteeism and unpaid work impairments.

Mortality, length of stay and cost of hospitalization among patients with systemic sclerosis: results from the National Inpatient Sample.

Objectives: To evaluate the hospitalizations and define the factors associated with in-hospital mortality, longer length of stay (LOS) and higher hospital costs among SSc hospitalizations. Methods: We used the National Inpatient Sample (2012-13) to identify adult hospitalizations with SSc, excluding patients with concomitant diagnosis of RA and systemic lupus. We calculated rates of hospitalization, in-hospital mortality, LOS and hospital costs. Factors associated with these outcomes were evaluated by univariate and backward stepwise multivariate logistic regression. Results: There were 9731 hospitalizations in the sample representing an estimated 48 655 hospitalizations nationwide with SSc (0.09%), and the inpatient mortality rate was 5%. Patients were predominantly older (mean age 63.2 years), female (82.2%) and Caucasian (71.5%). Infections were the most common primary diagnoses among SSc hospitalizations (17.4%) and among those who died (32.7%). Acute renal failure [adjusted odds ratio (aOR) = 4.3, 95% CI: 3.3, 5.6] and aspiration (aOR= 3.5, 95% CI: 2.5, 4.9) were strongly associated with in-hospital mortality. The median (interquartile range) LOS was 4 days (-2, 7), and the median (interquartile range) cost was $8885 (-5169, 15921). While hospital from the West region, acute renal failure, acute bowel obstruction and aspiration (aOR > 2.0 with P < 0.0001 for all) seem to predict higher cost of hospitalization, pulmonary fibrosis, myositis and any type of infection in addition to the same factors, except the West region (aOR > 2.0 with P < 0.0001 for all), were associated with longer LOS. Conclusion: Infections are currently the most common diagnoses among SSc hospitalizations and in-hospital deaths. This emphasizes the importance of being vigilant in prevention and early treatment of infections in SSc patients.

All-cause Healthcare Costs and Mortality in Patients with Systemic Sclerosis with Lung Involvement.

OBJECTIVE: Patients with systemic sclerosis (SSc) often develop interstitial lung disease (ILD) and/or pulmonary arterial hypertension (PAH). The effect of ILD and PAH on healthcare costs among patients with SSc is not well described. The objective of this analysis was to describe healthcare costs in patients with newly diagnosed SSc and SSc patients newly diagnosed with ILD and/or PAH in the United States. METHODS: This retrospective cohort analysis was conducted in the Truven Health MarketScan Commercial and Medicare Supplemental healthcare claims databases from 2003 to 2014. Based on International Classification of Diseases-9-Clinical Modification diagnosis codes on medical claims, patients were classified into 3 groups: incident SSc, SSc with incident ILD (SSc-ILD), and SSc with incident PAH (SSc-PAH). Patients were required to have continuous enrollment for 5 years to measure all-cause healthcare costs. Costs (adjusted to US$) were reported overall and by service type and year following diagnosis. Because of the overlap between groups, statistical comparisons were not conducted. RESULTS: There were 1957 patients with incident SSc, 219 with incident SSc-ILD, and 108 patients with incident SSc-PAH. Average (mean ± SD) all-cause healthcare costs over followup were higher for patients with incident SSc-ILD ($191,107 ± $322,193) or patients with incident SSc-PAH ($254,425 ± $240,497), compared to patients with incident SSc ($101,839 ± $167,155). Average annual costs over the 5-year period ranged from $18,513 to $23,268 for patients with incident SSc, from $31,285 to $55,446 for patients with incident SSc-ILD, and from $44,454 to $63,320 for patients with incident SSc-PAH. Costs tended to be the highest in the fifth year of followup. CONCLUSION: Among patients with SSc, ILD and PAH can result in substantial increases in healthcare costs.

Work productivity in systemic sclerosis, its economic burden and association with health-related quality of life.

Objective: To evaluate work productivity and its economic burden in SSc patients. Methods: Consecutive SSc patients enrolled in the Australian Scleroderma Cohort Study were mailed questionnaires assessing employment (Workers' Productivity and Activity Impairment Questionnaire and a custom-made questionnaire) and health-related quality of life (HRQoL) (36-item Short Form Health Survey and Patient-Reported Outcomes Measurement Information System 29). Linear regression methods were used to determine factors associated with work productivity. Results: Among 476 patients submitting responses, 55.2% <65 years of age were employed. Unemployed patients were older at the time of survey completion (57.1 vs 53.7 years; P < 0.001) and had longer disease duration from first SSc clinical manifestation (16.2 vs 14.9 years; P = 0.01) than employed patients. The mean age at unemployment onset was 13.2 years below the average Australian retirement age. Of those working in the week prior to completing the survey, 16.0% reported missing work (absenteeism) due to their SSc, accounting for 32.9% of their working week. Reduced productivity while at work (presenteeism) accounted for 22% of their working week. Annual costs per patient as a consequence of unemployment and reduced productivity equated to a total of AUD$67 595.40. Factors independently associated with reduced work productivity were presence of synovitis and sicca symptoms, while tertiary education protected against work impairment. Patients with low HRQoL scores also had low work productivity. Conclusion: SSc is associated with considerable unemployment and reduced productivity, which in turn is associated with a substantial economic burden and poor HRQoL. Raising awareness and identifying modifiable factors are possible ways of reducing this burden.

Humanistic and cost burden of systemic sclerosis: A review of the literature.

BACKGROUND: Systemic sclerosis (SSc), or systemic scleroderma, is a chronic multisystem autoimmune disease characterised by widespread vascular injury and progressive fibrosis of the skin and internal organs. Patients with SSc have decreased survival, with pulmonary involvement as the main cause of death. Current treatments for SSc manage a range of symptoms but not the cause of the disease. Our review describes the humanistic and cost burden of SSc. METHODS: A structured review of the literature was conducted, using predefined search strategies to search PubMed, Embase, and the Cochrane Library. Grey literature searches also were conducted. RESULTS: In total, 2226 articles were identified in the databases and 52 were included; an additional 10 sources were included from the grey literature. The review identified six studies reporting relevant cost estimates conducted in five different countries and four studies that assessed the humanistic burden of SSc. Total direct annual medical costs per patient for Europe varied from €3544 to €8452. For Canada, these costs were reported to be from Can$5038 to Can$10,673. In the United States, the total direct health care costs were reported to be US$17,365 to US$18,396. Different key drivers of direct costs were reported, including hospitalisations, outpatients, and medication. The total annual costs per patient were reported at Can$18,453 in Canada and varied from €11,074 to €22,459 in Europe. Indirect costs represented the largest component of the total costs. EQ-5D utility scores were lower for patients with SSc than those observed in the general population, with reported mean values of 0.49 and 0.68, respectively. The average value of the Health Assessment Questionnaire for patients with SSc was significantly higher than the control population (0.94), and the average value of the SF-36 was significantly lower than the control population: 49.99 for the physical dimension and 58.42 for the mental dimension. CONCLUSIONS: Overall, there is a paucity of information on the burden of SSc. Nonetheless, our review indicates that the quality of life of patients with SSc is considerably lower than that of the general population. In addition, SSc places a considerable economic burden on health care systems and society as a whole.

Social/economic costs and health-related quality of life in patients with scleroderma in Europe.

OBJECTIVE: The aim of this study was to determine the economic burden from a societal perspective and the health-related quality of life (HRQOL) of patients with systemic sclerosis (SSc; scleroderma) in Europe. METHODS: We conducted a cross-sectional study of patients with SSc (involving both localised and systemic sclerosis) from Germany, Italy, Spain, France, the UK, Hungary and Sweden. Data on demographic characteristics, healthcare resource utilisation, informal care, labour productivity losses and HRQOL were collected from the questionnaires completed by patients or their caregivers. HRQOL was measured with the EuroQol 5-domain (EQ-5D) questionnaire. RESULTS: A total of 589 patients completed the questionnaire. The rate of patients with localised scleroderma, limited cutan and diffuse cutan SSc were 28, 68 and 4 %, respectively. Average annual costs varied from country to country and ranged from € 4607 to € 30,797 (reference year: 2012). Estimated direct healthcare costs ranged from € 1413 to € 17,300; direct non-healthcare costs ranged from € 1875 to € 4684 and labour productivity losses ranged from € 1701 to € 14,444. The mean EQ-5D index score for adult SSc patients varied from 0.49 to 0.75 and the mean EQ-5D visual analogue scale score was between 58.72 and 65.86. CONCLUSION: The main strengths of this study lie in our bottom-up approach to costing and our evaluation of SSs patients from a broad societal perspective. This type of analysis is very unusual in the international literature on rare diseases in comparison with other illnesses. We concluded that SSc patients incur considerable societal costs and experience substantial deterioration in HRQOL.

Cost savings with a new screening algorithm for pulmonary arterial hypertension in systemic sclerosis.

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 .

The indirect costs of systemic autoimmune diseases, systemic lupus erythematosus, systemic sclerosis and sarcoidosis: a summary of 2012 real-life data from the Social Insurance Institution in Poland.

Systemic lupus erythematosus, systemic sclerosis and sarcoidosis are three different autoimmune systemic diseases that generate a significant burden to society due to treatment costs and also those caused by a work disability or absenteeism among patients. Relevant 2012 data referring to the three components of absenteeism produced by autoimmune systemic diseases, sick leave, short-term and long-term work disability, were obtained from the Social Insurance Institution in Poland (PSII). By applying the Human Capital Approach using gross domestic product per capita, gross value added per worker and gross income per worker in 2012, total indirect costs for the diseases were calculated. All costs were presented in euros and were valid for 2012. The PSII recorded 1600 patients with systemic lupus erythematosus, 500 patients with systemic sclerosis and 2700 patients with sarcoidosis in the 2012 - total indirect costs were as high as 7,260,595, 2,268,571 and 4,027,575 EUR, respectively. Costs were estimated using gross domestic product per capita; 17,485,412, 5,463,312 and 9,699,455 EUR, accordingly, calculated using gross value added per worker and 5,346,933, 1,670,648 and 2,966,034 EUR estimated using gross income per worker, respectively. Considering only data on absenteeism gathered by the PSII we can conclude that the three autoimmune systemic diseases bore great indirect costs. Their social burden for Poland could be even greater when considering presenteeism as well as other components of absenteeism such as loss of unpaid work, a gray economy or loss of leisure time.

Factors influencing the occupational trajectory of patients with systemic sclerosis: a qualitative study.

OBJECTIVES: To describe, from the patient's point of view, the factors influencing the occupational trajectory of patients with systemic sclerosis (SSc). METHODS: This was a qualitative study designed using grounded theory with constant comparison. Data were collected through semi-structured interviews with 14 patients who fulfilled the American College of Rheumatology or Leroy-Medsger criteria for SSc. RESULTS: Based on our interviews, we found that the occupational trajectory of patients with SSc is influenced by the continuous interplay between four groups of factors. The first group concerns the values patients attribute to work, including identity, normality, financial value, social contact, and structure. The meaning of these values and how they relate to each other underlies the desire to work. A second group of factors is those influencing the balance between daily life, work participation, and medical condition (e.g. job content, flexibility in organising work, and the willingness to ask for accommodations at work). The occupational trajectory is also influenced by external factors, including availability of support, know-ledge of the disease, pressure to work, contact with medical professionals, and existing regulations and the patient's knowledge about them. Finally, the occupational trajectory is influenced by personal factors, including socio-demographics, psychological assets, and disease- and work-related personal factors. CONCLUSIONS: The decisions patients with SSc take concerning work depend on an interplay between many factors and, especially, on the patients' personal interpretation of these factors. These need to be taken into account when helping patients with SSc determine their occupational trajectory.

The economic burden and health-related quality of life associated with systemic sclerosis in France.

OBJECTIVES: To provide data on the economic burden and health-related quality of life (HRQoL) associated with systemic sclerosis (SSc) in France and to raise awareness of the repercussions of this disease for patients and caregivers and on the health and social care system. METHOD: A cross-sectional study was carried out on 147 patients recruited through the Association des Sclérodermiques de France (ASF), the French association for SSc patients. Data on the patients' use of resources were obtained retrospectively from an online questionnaire and costs were estimated by a bottom-up approach. The HRQoL patients and caregivers was assessed with the five-level EURQol-5 Dimension (EQ-5D-5L) health questionnaire. RESULTS: The average annual cost of SSc was estimated at EUR 22,459 per patient. Direct healthcare costs amounted to EUR 8452, direct non-healthcare formal costs to EUR 1606, direct non-healthcare informal costs to EUR 1875, and indirect costs resulting from patients' absence from the labour market to EUR 10,526. The main contributors to SSc costs were hospitalizations and early retirement. Mean EQ-5D utility scores were 0.49 for patients and 0.66 for caregivers. CONCLUSIONS: Although SSc is a rare disease, its economic burden from a societal perspective is substantial and the consequences for HRQoL are significant for both patients and caregivers in France, underscoring the need to develop tailored policies targeted at improving patients' care and reducing the long-term impact of SSc.

Social economic costs and health-related quality of life in patients with systemic sclerosis in Spain.

OBJECTIVE: Cost-of-illness analysis is the main method of providing an overall vision of the economic impact of a disease. Such studies have been used to set priorities for health care policies and inform resource allocation. The aim of this study was to determine the economic burden from a societal perspective and health-related quality of life (HRQOL) of patients with systemic sclerosis (SSc; scleroderma) in Spain. METHODS: We conducted a cross-sectional, retrospective study of 147 patients with SSc. Data on demographic characteristics, health resource utilization, informal care, labor productivity losses, and HRQOL were collected from the questionnaires completed by patients or their caregivers. HRQOL was measured with the EuroQol 5-domain (EQ-5D) questionnaire. RESULTS: The average annual cost for an SSc patient was 21,042; the reference year for unit prices was 2011. Direct health care costs amounted to 8,235, direct non-health care costs were 5,503, and loss of labor productivity was 7,303. The largest expenditures were early retirement, informal care, and medication. There are sharp differences in the estimated cost according to the Barthel Index score observed. The mean EQ-5D score for SSc patients was 0.68 and the mean EQ-5D visual analog scale score was 64. CONCLUSION: The main strengths of this study lie in our bottom-up approach to costing and in the evaluation of SSc patients from a broad perspective (societal costs). This type of analysis is very scarce in international literature for rare diseases in comparison with other illnesses. We conclude that SSc patients incur considerable societal costs and experience substantial deterioration in HRQOL.

A comparison of the measurement properties and estimation of minimal important differences of the EQ-5D and SF-6D utility measures in patients with systemic sclerosis.

OBJECTIVES: To compare measurement properties of the EQ-5D and SF-6D utility measures, to assess the association and agreement between these measures and to estimate minimal important differences (MID) in patients with systemic sclerosis (SSc). METHODS: Both measures were assessed twice in an observational prospective design over a 12-month period (n=211). Spearman's rank correlation between the EQ-5D and SF-6D was calculated at baseline. Agreement was assessed using Lin's concordance coefficient (LCC) and a Bland-Altman plot. MIDs were estimated using three anchors; the global rating of change item (SF-36) and changes on the Health Assessment Questionnaire-Disability Index (HAQ-DI) of ≥0.14 and ≥0.22. RESULTS: At baseline, the mean EQ-5D and SF-6D scores were 0.64 (SD=0.25) and 0.65 (SD=0.11), respectively. The correlation between EQ-D and SF-6D scores was r=0.74. Agreement was moderate (LCC=0.49), and the Bland-Altman plot showed a mean difference of 0.003 but wide limits of agreement (-0.38 to 0.39) and a structural bias for lower scores. The mean MID estimate for the EQ-5D was 0.08 for the improved subgroup, and -0.13 for the deteriorated subgroup. For the SF-6D, the MID estimate was 0.05 for the improved and -0.04 for the deteriorated subgroup. CONCLUSIONS: Although there was a marked correlation between the measures, the moderate agreement implies that EQ-5D and SF-6D scores cannot be used interchangeably. The MID estimates we provided can be used to calculate sample sizes for clinical trials involving SSc patients, and in interpreting the relevance and importance of treatment effects.