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Influence of interstitial lung disease on outcome in systemic sclerosis: a population-based historical cohort study.

ABSTRACT: Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and a major cause of SSc-related deaths. This study aimed to determine the influence of ILD on SSc in a population-based historical cohort study. The hypothesis was that patients with SSc who develop ILD have increased morbidity and mortality when compared with patients with SSc without ILD.Using the record linkage system of the Rochester Epidemiology Project in Olmsted County, Minnesota, this study identified the incidence of SSc between 1980 and 2010 and point prevalence on December 31, 2010 and determined the progression of organ involvement and its influence on outcome.During the 30-year interval, we identified 64 incident cases of SSc: 57 women and seven men, median age 49.1 years (interquartile range [IQR], 39.8-67.6 years). There were 43 prevalent cases. ILD occurred in 19 cases, usually after the diagnosis of SSc (median, 2 years; IQR, 0-10 years), with only three cases occurring 6 to 24 months beforehand. Pulmonary arterial hypertension (PAH) was diagnosed in 14 cases, heart failure in 27 cases, and chronic kidney disease (CKD) in 21 cases. Seventeen patients died during the study period, with a median survival time after diagnosis of 22.9 years. ILD, PAH, and CKD were associated with an increased risk of death.The incidence of ILD associated with SSc was relatively low in this population-based cohort. ILD appeared to be a contributing factor to mortality. Other factors, including age, PAH, and CKD, were also associated with poor outcome.

SUBMITTER: Bauer PR

PROVIDER: S-EPMC3734889 | biostudies-other | 2013 Aug

REPOSITORIES: biostudies-other

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Predictors of lung function test severity and outcome in systemic sclerosis-associated interstitial lung disease.

Project description:Systemic sclerosis-related interstitial lung disease (SSc-ILD) is the leading cause of death in SSc. In this study, we aimed to describe the baseline severity and evolution of forced vital capacity (FVC) and diffusing capacity for carbon monoxide (DLCO) in patients with SSc-ILD and to assess the baseline clinical, biological and high-resolution CT scan (HRCT) predictors of this evolution. Baseline and serial FVC and DLCO were collected in 75 SSc-ILD patients followed during 6.4±4.2 years (n = 557 individual data). FVC and DLCO evolution was modelled using a linear mixed model with random effect. During follow-up, FVC was stable while DLCO significantly decreased (-1.5±0.3%/year (p<0.0001). Baseline NYHA functional class III/IV, extensive SSc-ILD on HRCT and DLCO<80% were associated with a lower baseline FVC. Absence of digital ulcers extensive SSc-ILD, and FVC<80% and were associated with a lower baseline DLCO. Presence or history of digital ulcers and presence of pulmonary hypertension at baseline or during follow-up were associated with a faster decline of DLCO overtime. Neither age, gender, subtype of SSc nor specificity of autoantibodies were associated with baseline severity or outcome of lung function tests. In this SSc-ILD population, FVC was therefore stable while DLCO significantly declined over time. ILD extension was associated with baseline FVC and DLCO but not with their evolution. Presence or history of digital ulcers and pulmonary hypertension were predictors of a faster decline of DLCO over time.

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Project description:Background and objectiveThe study aimed to evaluate the direct and indirect costs of systemic sclerosis (SSc) in cases with and without interstitial lung disease (ILD).MethodsCases diagnosed with SSc (2002-2015) were identified in the Danish National Patient Registry. Cases were matched 1:4 with non-SSc controls from the general population. Data on costs were obtained from national databases. Excess cost was estimated as the annual cost per case subtracting the costs of the control.ResultsWe identified 1869 cases and 7463 controls. Total excess cost (direct healthcare, elderly care and indirect costs) in the SSc-ILD cohort was €29,725, and €17,905 in the non-ILD SSc cohort. In- and out-patient contacts and forgone earnings were the key drivers of costs in both cohorts. Healthcare costs were higher before and after the diagnosis compared with the controls. Men incurred higher excess healthcare costs than women. Hospitalization and outpatient services were the key drivers of the gender-associated differences. Income from employment decreased more rapidly after diagnosis in the SSc-ILD cohort than in the non-ILD SSc cohort. Public transfer income increased after diagnosis, with the most pronounced difference in the SSc-ILD cohort. Disability pension was the key driver of public transfer income.ConclusionSSc is associated with a significant individual and societal burden that is evident several years before and after the diagnosis. Total excess costs are higher in SSc-ILD than in the non-ILD SSc underlining the severity of pulmonary involvement. Initiatives to maintain work ability and to reduce hospital admissions may reduce the economic burden of SSc.

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Project description:Interstitial lung disease (ILD) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). Although a large proportion of SSc patients have only limited interstitial involvement with an indolent course, in a significant minority ILD is progressive, requiring prompt treatment and careful monitoring. One of the main challenges for the clinician treating this highly variable disease is the early identification of patients at risk of progressive ILD, while avoiding potentially toxic treatments in those whose disease is inherently stable. Easily available and repeatable biomarkers that allow estimation of the risk of ILD progression and early response to treatment are highly desirable. In this paper, we review the evidence for circulating biomarkers with potential roles in diagnosis, monitoring of disease activity, or determining prognosis. Peripheral blood biomarkers offer the advantages of being readily obtained, non-invasive, and serially monitored. Several possible candidates have emerged from studies performed so far, including SP-D, KL-6, and CCL18. Presently however, there are few prospective studies evaluating the predictive ability of prospective biomarkers after adjustment for disease severity. Future carefully designed, prospective studies of well characterised patients with ILD, with optimal definition of disease severity and outcome measures are needed.

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Interstitial lung disease associated with systemic sclerosis (SSc-ILD).

Project description:BackgroundSystemic sclerosis (SSc) is a rare connective tissue disease with a heterogeneous clinical course. Interstitial lung disease (ILD) is a common manifestation of SSc and a leading cause of death.Main bodyAll patients newly diagnosed with SSc should receive a comprehensive clinical evaluation, including assessment of respiratory symptoms, a high-resolution computed tomography (HRCT) scan of the chest, and pulmonary function tests. ILD can develop in any patient with SSc, including those with pulmonary hypertension, but the risk is increased in those with diffuse (rather than limited) cutaneous SSc, those with anti-Scl-70/anti-topoisomerase I antibody, and in the absence of anti-centromere antibody. While it can occur at any time, the risk of developing ILD is greatest early in the course of SSc, so patients should be monitored closely in the first few years after diagnosis. An increased extent of lung fibrosis on HRCT and a low forced vital capacity (FVC) are predictors of early mortality. While not all patients will require treatment, current approaches to the treatment of progressive SSc-ILD focus on immunosuppressant therapies, including cyclophosphamide and mycophenolate mofetil. In patients with severe and/or rapidly progressive disease, both haematopoietic stem cell transplantation (HSCT) and lung transplantation have been successfully used. A number of medications, including the two drugs approved for the treatment of idiopathic pulmonary fibrosis (IPF), are under active investigation as potential new therapies for SSc-ILD.ConclusionsPhysicians managing patients with SSc should maintain a high level of suspicion and regularly monitor for ILD, particularly in the first few years after diagnosis.

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Systemic Sclerosis-Associated Interstitial Lung Disease: Lessons from Clinical Trials, Outcome Measures, and Future Study Design.

Project description:Pulmonary involvement including interstitial lung disease (ILD) is the leading cause of mortality in patients with systemic sclerosis (scleroderma; SSc). This article reviews the current evidence based medicine regarding available therapies for SSc-ILD ; discusses the lessons learned from recent SSc-ILD randomized controlled trials (RCTs); and proposes outcome measures and recommendations for design of future RCTs for SSc-ILD.

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Disease frequency, patient characteristics, comorbidity outcomes and immunosuppressive therapy in systemic sclerosis and systemic sclerosis-associated interstitial lung disease: a US cohort study.

Project description:ObjectivesTo investigate prevalence estimates and incidence rates (IRs) for SSc and SSc-associated interstitial lung disease (SSc-ILD) cohorts and describe patient characteristics, immunosuppressive therapy (IST) and comorbid outcomes among incident SSc and SSc-ILD cohorts.MethodsData were obtained from the US IBM MarketScan (2008-2017) claims database using algorithms developed with expert consultation. For the SSc cohort, newly diagnosed patients (aged ≥18 years) had one or more diagnostic claim for SSc. For the SSc-ILD cohort, patients had an additional ILD claim. Sensitivity analyses using two or more claims or alternative ILD diagnostic codes were also conducted.ResultsWhen requiring one or more diagnostic claim, the prevalence of SSc and SSc-ILD per 100 000 persons was 72.1 and 19.0. The IR for SSc and SSc-ILD per 100 000 person-years was 18.3 and 4.3. Sensitivity analyses requiring two or more claims yielded much lower prevalence (SSc: 41.5; SSc-ILD: 13.3) and IR (SSc: 8.8; SSc-ILD: 1.6) estimates. Patients with SSc-ILD were older, with increased comorbidities and diagnostic procedures at baseline. MTX and MMF were the most common ISTs; 12.7% of the SSc-ILD cohort received therapy at baseline vs 8.2% for SSc. A total of 42.5% and 45.0% of the SSc and SSc-ILD cohorts, respectively, started a stable IST regimen and 21.7% and 19.4% of these had an escalation. Skin disorders were the most common comorbid outcome in both cohorts during follow-up.ConclusionsSSc, with or without associated ILD, is a rare disease in the US. Newly diagnosed patients with SSc-ILD had received more IST and had more comorbidities compared with newly diagnosed SSc.

| S-EPMC8023990 | biostudies-literature

Systemic Sclerosis Associated Interstitial Lung Disease: New Directions in Disease Management.

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