Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management

Chowaniec, Małgorzata; Skoczyńska, Marta; Sokolik, Renata; Wiland, Piotr

doi:10.5114/reum.2018.77977

Cited by 24 publications

(18 citation statements)

References 42 publications

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“…Severe PAH was defined based on presence of four of the following PAH severity criteria: WHO-FC IV, pericardial effusion, six-minute walk distance (6MWD) < 300 m, RAP on RHC of > 15 mmHg and CI of < 2 L/min/m 2 [19]. Mild ILD was defined by characteristic fibrotic changes on high-resolution computed tomography (HRCT) lung (< 10% abnormal lung involvement) and a forced vital capacity of > 70% on respiratory function tests [17, 20]. Monotherapy is treatment with a single PAH specific therapy (endothelin receptor antagonists (ERA), phosphodiesterase-5-inhibitors (PDE5) or prostacyclin analogues) and was prescribed at the discretion of the managing physician(s), while combination therapy is treatment with more than one specific PAH agent from different classes at one time.…”

Section: Methodsmentioning

confidence: 99%

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

et al. 2019

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BackgroundTo quantify the financial cost of pulmonary arterial hypertension (PAH) in systemic sclerosis (SSc).MethodsHealthcare 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.ResultsTotal 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% utilizing a healthcare service within the first 12 months post PAH diagnosis with an associated cost per patient of AUD$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.ConclusionsDespite 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.

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Section: Methodsmentioning

confidence: 99%

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

et al. 2019

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“…Despite recommendations to perform these assessments (as well as 6MWT) at regular intervals in patients with idiopathic pulmonary fibrosis (IPF),97 there are no formal guidelines for monitoring progression in SSc-ILD. Most clinicians would perform PFTs every 3–6 months,30 whereas opinions on the optimal interval between HRCT assessments are variable. Table 2 shows our suggestions for monitoring patients with SSc-ILD, including those with early disease.…”

Section: Monitoring and Predicting The Progression Of Ild In Patientsmentioning

confidence: 99%

<p>Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention</p>

Fischer

Patel

Volkmann

2019

OARRR

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Systemic sclerosis (SSc) is a progressive and often devastating disease characterized by autoimmune dysfunction, vasculopathy, and fibrosis. Interstitial lung disease (ILD) is identified in the majority of patients with SSc and is the leading cause of SSc-related mortality. Although clinical manifestations and ILD severity vary among patients, lung function typically declines to the greatest extent during the first 3–4 years after disease onset. We aim to provide an overview of SSc-associated ILD (SSc-ILD) with a focus on current and emerging tools for early diagnosis of ILD and current and novel treatments under investigation. Early detection of ILD provides the opportunity for early therapeutic intervention, which could improve patient outcomes. Thoracic high-resolution computed tomography is the most effective method of identifying ILD in patients with SSc; it enables detection of mild lung abnormalities and plays an important role in monitoring disease progression. Cyclophosphamide and mycophenolate mofetil are the most commonly prescribed treatments for SSc-ILD. Recently, nintedanib (an antifibrotic) was approved by the Food and Drug Administration for patients with SSc-ILD; it is indicated for slowing the rate of decline in pulmonary function. However, there is a need for additional effective and well-tolerated disease-modifying therapy. Ongoing studies are evaluating other antifibrotics and novel agents. We envision that early detection of lung involvement, combined with the emergence and integration of novel therapies, will lead to improved outcomes in patients with SSc-ILD.

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“…Considering that ILD represents a major cause of mortality in SSc [2], preventing the progression of respiratory impairment is of utmost importance to improve life expectancy of SSc patients and it has been the target of several investigational studies. Pulmonary function tests (PFTs) were always, although variably, included among the assessed outcomes, and, in clinical practice, PFTs have an established role in screening and follow-up of SSc patients [3].…”

Section: Introductionmentioning

confidence: 99%

Lung function is associated with minimal EQ-5D changes over time in patients with systemic sclerosis

et al. 2020

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In systemic sclerosis (SSc) therapeutic efforts are often directed to prevent progressive respiratory impairment, but it is unclear to what extent changes in pulmonary function tests (PFTs) are associated with health-related quality of life (HRQoL). The aim of our study is to evaluate how modifications in PFTs contribute to longitudinal variations in HRQoL, assessed through the multidimensional questionnaire EQ-5D, in patients with SSc. We included SSc patients with forced vital capacity (FVC%), diffusing capacity of the lungs for carbon monoxide (DLCO%) and EQ-5D assessed in at least two visits. The EQ-5D consists of two parts, a utility score ranging from − 0.59 to 1, and a 0-100 Visual Analogue Scale (VAS). Higher values represent better health. The association between changes in FVC% and DLCO%, and evolution of EQ-5D over time, was investigated using generalized estimating equations. Three hundred seventy-eight patients were included, accounting for a total of 1619 measurements. The models showed that improvement in FVC% is significantly associated with increase in both utility score (β = 0.001; 95% CI 0.000 to 0.002; p = 0.003) and VAS over time (β = 0.188; 95% CI 0.111 to 0.264; p < 0.001). Moreover, improvement in DLCO% is longitudinally associated with increase in utility score (β = 0.001; 95% CI 0.000 to 0.002; p = 0.038), while the results for VAS were non-significant (β = 0.020; 95% CI-0.079 to 0.120; p = 0.690). We show that change in PFTs has a significant, although minor, impact on HRQoL as measured by EQ-5D in SSc. Key Points • In patients with SSc, changes in PFTs have a significant, although minor, impact on HRQoL. • In patients with SSc-ILD, the perception of HRQoL is nearly not influenced by changes in pulmonary function. • The use of generic questionnaires might not be sensitive enough to evaluate the impact on quality of life of therapies targeting specific SSc manifestations.

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Interstitial lung disease in systemic sclerosis: challenges in early diagnosis and management

Cited by 24 publications

References 42 publications

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

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

<p>Interstitial Lung Disease in Systemic Sclerosis: Focus on Early Detection and Intervention</p>

Lung function is associated with minimal EQ-5D changes over time in patients with systemic sclerosis

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