Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease

Caron, Melissa; Hoa, Sabrina; Hudson, Marie; Schwartzman, Kevin; Steele, Russell

doi:10.1183/16000617.0102-2017

Cited by 68 publications

(60 citation statements)

References 173 publications

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“…In reality, at the moment, the treatment of ILD-SSc has produced conflicting results, despite the large number of compounds studied [30]. The lack of significant results in clinical trials can be explained by the absence of validated primary outcomes (e.g., PFTs parameters [31]), but a role could be played by the frequent absence of stratification according to the HRCT pattern. A prevalent GGO involvement could be an expression of DA and it might benefit from aggressive immunosuppression, whereas in the presence of HC areas or even a UIP pattern, this treatment could be detrimental, as reported for idiopathic pulmonary fibrosis [32].…”

Section: Discussionmentioning

confidence: 99%

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Sambataro

Pignataro³

et al. 2020

Diagnostics

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Interstitial lung disease (ILD) is the main cause of death in systemic sclerosis (SSc) patients. Usually, patients have lung involvement characterized by ground glass opacities (GGOs), but honeycombing (HC) is also possible. The Wells score is a semi-quantitative index, which is able to assess ILD by distinguishing its main components. The aim of this work is to evaluate the Wells score in relation to the disease activity (DA) index. We enrolled 40 consecutive SSc-ILD patients (26 diffuse cutaneous form, dcSSc, and 14 limited form, lcSSc). All patients were evaluated by the European Scleroderma Study Group (ESSG) index, high-resolution computed tomography, transthoracic echocardiogram, pulmonary function tests (PTSs), and nailfold videocapillaroscopy for the number of microhemorrhages (NEMO) score. In our study, the total extent of ILD (TE-ILD), fibrosis and GGOs correlated with dyspnea (p = 0.03, 0.01 and 0.01 respectively), but not with the ESSG index. Considering only the dcSSc patients, TE-ILD and GGOs correlated with the ESSG index (r = 0.5 p = 0.009), while fibrosis grade correlated with disease duration and systolic pulmonary artery pressure. In conclusion, our data suggest that GGO correlates with DA, while fibrosis may be a sign of disease damage. The quantification of pulmonary involvement using the Wells score can be a useful tool for assessing the appropriate treatment in SSc patients.

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Section: Discussionmentioning

confidence: 99%

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Sambataro

Pignataro³

et al. 2020

Diagnostics

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“…Moreover, new drugsrituximab [45,46], mycophenolate [47], their combination [48], and nintedanib [49] have the potential to provide an effective therapy for ILD. These therapies have been shown to improve parameters of pulmonary function that are related to prognosis, such as DLCO, DLCO/ FVC and TLC [45,50,51], but to date no study has actually demonstrated improved survival in patients treated with immunosuppressive agents. Hence, there is a need for new parameters that better predict long time survival under immunosuppression [52].…”

Section: Discussionmentioning

confidence: 99%

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

et al. 2019

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Background: Systemic sclerosis (SSc) is a severe rheumatic disease of the interstitial tissue, in which heart and lung involvement can lead to disease-specific mortality. Our study tests the hypothesis that in addition to established prognostic factors, cardiopulmonary exercise testing (CPET) parameters, particularly peak oxygen uptake (peakVO 2) and ventilation/carbon dioxide (VE/VCO 2)-slope, can predict survival in patients with SSc. Subjects and methods: We retrospectively assessed 210 patients (80.9% female) in 6 centres over 10 years with pulmonary testing and CPET. Survival was analysed with Cox regression analysis (adjusted for age and gender) by age, comorbidity (Charlson-Index), body weight, body-mass index, extensive interstitial lung disease, pulmonary artery pressure (measured by echocardiography and invasively), and haemodynamic, pulmonary and CPET parameters. Results: Five-and ten-year survival of SSc patients was 93.8 and 86.9%, respectively. There was no difference in survival between patients with diffuse (dcSSc) and limited cutaneous manifestation (lcSSc; p = 0.3). Pulmonary and CPET parameters were significantly impaired. Prognosis was worst for patients with pulmonary hypertension (p = 0.007), 6-min walking distance < 413 m (p = 0.003), peakVO 2 < 15.6 mL•kg − 1 •min − 1 , and VE/VCO 2-slope > 35. Age (hazard ratio HR = 1.23; 95% confidence interval CI: 1.14;1.41), VE/VCO 2-slope (HR = 0.9; CI 0.82;0.98), diffusion capacity (Krogh factor, HR = 0.92; CI 0.86;0.98), forced vital capacity (FVC, HR = 0.91; CI 0.86;0.96), and peakVO 2 (HR = 0.87; CI 0.81;0.94) were significantly linked to survival in multivariate analyses (Harrell's C = 0.95). Summary: This is the first large study with SSc patients that demonstrates the prognostic value of peakVO 2 < 15.6 mL•kg − 1 •min − 1 (< 64.5% of predicted peakVO 2) and VE/VCO 2-slope > 35.

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“…Due to the heterogeneity of the disease, SSc represents a major clinical challenge for both physicians and patients [2]. In addition to disfiguring skin involvement, SSc can affect multiple organ systems, including the lungs [3]. The clinical course is variable, but organ manifestations frequently occur early in the disease [4].…”

Section: Introductionmentioning

confidence: 99%

Predictors of progression in systemic sclerosis patients with interstitial lung disease

Assassi²,

et al. 2020

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Systemic sclerosis (SSc) is a systemic autoimmune disease affecting multiple organ systems, including the lungs. Interstitial lung disease (ILD) is the leading cause of death in SSc.There are no valid biomarkers to predict the occurrence of SSc-ILD, although auto-antibodies against anti-topoisomerase I and several inflammatory markers are candidate biomarkers that need further evaluation. Chest auscultation, presence of shortness of breath and pulmonary function testing are important diagnostic tools, but lack sensitivity to detect early ILD. Baseline screening with high-resolution computed tomography (HRCT) is therefore necessary to confirm an SSc-ILD diagnosis. Once diagnosed with SSc-ILD, patients' clinical courses are variable and difficult to predict, although certain patient characteristics and biomarkers are associated with disease progression. It is important to monitor patients with SSc-ILD for signs of disease progression, although there is no consensus about which diagnostic tools to use or how often monitoring should occur. In this article, we review methods used to define and predict disease progression in SSc-ILD.There is no valid definition of SSc-ILD disease progression, but we suggest that either a decline in forced vital capacity (FVC) from baseline of ≥10%, or a decline in FVC of 5–9% in association with a decline in diffusing capacity of the lung for carbon monoxide of ≥15% represents progression. An increase in the radiographic extent of ILD on HRCT imaging would also signify progression. A time period of 1–2 years is generally used for this definition, but a decline over a longer time period may also reflect clinically relevant disease progression.

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Pulmonary function tests as outcomes for systemic sclerosis interstitial lung disease

Cited by 68 publications

References 173 publications

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Quantification of Ground Glass Opacities Can Be Useful to Describe Disease Activity in Systemic Sclerosis

Prognostic value of cardiopulmonary exercise testing in patients with systemic sclerosis

Predictors of progression in systemic sclerosis patients with interstitial lung disease

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