Esophageal Dilation and Other Clinical Factors Associated with Pulmonary Function Decline in Patients with Systemic Sclerosis

2021 ◽  
pp. jrheum.210533
Author(s):  
Kimberly Showalter ◽  
Aileen Hoffmann ◽  
Carrie Richardson ◽  
David Aaby ◽  
Jungwha Lee ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Function ◽  
Pulmonary Function Tests ◽  
Esophageal Dilation ◽  
Clinical Factors ◽  
High Resolution Computed Tomography ◽  
Mixed Effect ◽  
Mixed Effect Models ◽  
Clinically Significant

Objective To identify clinical factors, including esophageal dilation on chest high-resolution computed tomography (HRCT), that are associated with pulmonary function decline in patients with systemic sclerosis (SSc). Methods Patients fulfilled 2013 SSc criteria and had ≥1 HRCT and ≥2 pulmonary function tests (PFTs). According to published methods, widest esophageal diameter (WED) and radiographic interstitial lung disease (ILD) were assessed, and WED was dichotomized as dilated (≥19mm) vs. not dilated (<19mm). Clinically meaningful PFT decline was defined as %-predicted change in forced vital capacity (FVC) ≥5 and/or diffusion capacity for carbon monoxide (DLCO) ≥15. Linear mixed effect models were used to model PFT change over time. Results 138 SSc patients met study criteria: 100 (72%) had radiographic ILD; 49 (35%) demonstrated FVC decline (median follow-up 2.9y). Patients with Scl-70 autoantibodies had 5- year %-predicted FVC decline (-6.3; 95% CI -9.9, -2.8), while patients without Scl-70 autoantibodies demonstrated 5-year FVC stability (+1.78; 95% CI -0.6, 4.15). Esophageal diameter did not distinguish between those with vs. without FVC decline. Patients with esophageal dilation had statistically significant 5-year %-predicted DLCO decline (-5.6; 95% CI - 10.0, -1.2), but this decline was unlikely clinically significant. Similar results were observed in sub-analysis of patients with radiographic ILD. Conclusion In patients with SSc, Scl-70 positivity is a risk factor for %-predicted FVC decline at five years. Esophageal dilation on HRCT was associated with a minimal, non-clinically significant decline in DLCO and no change in FVC during 5-year follow-up. These results have prognostic implications for SSc-ILD patients with esophageal dilation.

scholarly journals Serum IGFBP-2 in systemic sclerosis as a prognostic factor of lung dysfunction

2021 ◽  
Vol 11 (1) ◽  
Author(s):  
Julien Guiot ◽  
Makon-Sébastien Njock ◽  
Béatrice André ◽  
Fanny Gester ◽  
Monique Henket ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Prognostic Factor ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Serum Levels ◽  
Roc Curve Analysis ◽  
Significant Deterioration ◽  
Lung Dysfunction

AbstractSystemic sclerosis (SSc) is a rare connective tissue disease associated with rapid evolving interstitial lung disease (ILD), driving its mortality. Specific biomarkers associated with the progression of this lung disease are highly needed. We aimed to identify specific biomarkers of SSc-ILD to predict the evolution of the disease. For this, we compared prospectively serum levels of several biomarkers associated with lung fibrosis in SSc patients (n = 102), among which SSc-no ILD (n = 63) and SSc-ILD (n = 39), compared to healthy subjects (HS) (n = 39). We also performed a longitudinal study in a subgroup of 28 patients analyzing biomarkers variations and pulmonary function tests over a period of 2 years. Serum level of IGFBP-2 was significantly increased in SSc patients compared to HS, and negatively correlated with pulmonary function (assessed by carbon monoxide transfer coefficient (KCO)) (r = − 0.29, p < 0.01). Two-year longitudinal analysis in a subgroup of 28 SSc patients determined that IGFBP-2 variation was positively correlated with KCO at 2-year follow-up (r = 0.6, p < 0.001). SSc patients with a lower variation of IGFBP-2 (less than 22%) presented significant deterioration of pulmonary function at 2-year follow-up (p < 0.01). ROC curve analysis enabled us to identify that baseline IGFBP-2 > 105 ng/ml was associated with a poor outcome (KCO < 70% predicted) at 2-year follow-up (AUC = 0.75, p < 0.05). We showed for the first time that serum levels of IGFBP-2 might be a prognostic factor of the development of SSc-ILD.

Effect of Bosentan on Systemic Sclerosis-associated Interstitial Lung Disease Ineligible for Cyclophosphamide Therapy: A Prospective Open-label Study

2011 ◽  
Vol 38 (10) ◽  
pp. 2186-2192 ◽  
Author(s):  
YOSHIAKI FURUYA ◽  
MASATAKA KUWANA
Keyword(s):  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
High Dose ◽  
Open Label ◽  
High Resolution Computed Tomography ◽  
Open Label Study ◽  
Label Study

Objective.To evaluate the clinical benefits of the endothelin receptor antagonist bosentan on interstitial lung disease (ILD) in patients with systemic sclerosis (SSc) who are ineligible for cyclophosphamide (CYC) therapy.Methods.In this prospective open-label study, 9 patients with SSc and ILD received bosentan for 24 months. The main reasons for avoiding CYC included severely impaired lung function, long disease duration, and relapse after CYC treatment. Pulmonary function tests and Doppler echocardiograms were evaluated every 6 months, and high-resolution computed tomography (HRCT) was performed every 12 months. For an extended survival analysis, 17 historical controls who met the inclusion criteria at referral and had not used any immunosuppressive or antifibrotic agents thereafter were selected from the SSc database.Results.Two patients did not finish the study; one developed vasculitis requiring high-dose corticosteroids and another died of bacterial pneumonia. The remaining 7 patients tolerated bosentan and completed the study period. There were trends toward mildly reduced forced vital capacity, total lung capacity, and diffusing capacity for carbon monoxide over time. Two patients developed pulmonary hypertension during the 24-month period. HRCT scores for ground-glass opacity, pulmonary fibrosis, and honeycomb cysts gradually increased. In the extended study, there was no difference in cumulative survival rate between the bosentan-treated and historical control groups.Conclusion.The gradual worsening of pulmonary function and HRCT findings in patients treated with bosentan was consistent with the natural course of SSc-associated ILD. This study does not support the use of bosentan for SSc-associated ILD even when CYC treatment is inadvisable.

scholarly journals High-Resolution Computed Tomography: Lights and Shadows in Improving Care for SSc-ILD Patients

Diagnostics ◽  
2021 ◽  
Vol 11 (11) ◽  
pp. 1960
Author(s):  
Barbara Ruaro ◽  
Elisa Baratella ◽  
Paola Confalonieri ◽  
Barbara Wade ◽  
Cristina Marrocchio ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
High Resolution ◽  
Pulmonary Function Tests ◽  
High Resolution Computed Tomography ◽  
Mild Disease ◽  
Treatment Regimens ◽  
Assessment And Monitoring ◽  
Diagnosis And Classification

The diagnosis and classification of systemic sclerosis-associated interstitial lung disease (SSc-ILD) is essential to improve the prognosis of systemic sclerosis (SSc) patients. The risk-stratification of disease severity and follow-up requires a multidisciplinary approach, integrating high-resolution computed tomography (HRTC) of the lung, pulmonary function tests (PFT), along with clinical and symptomatic evaluations. The use of HRCT in detecting SSc-ILD is not so much based on a definitive validation, but rather reflects the widespread clinician recognition of dissatisfaction with other modalities. However, due to the heterogeneity of SSc-ILD and the potential absence of symptoms in early or mild disease, it is prudent to consider as many parameters as possible in the assessment and monitoring of newly diagnosed patients. An early diagnosis meets the primary goal, i.e., the prevention of disease progression. The current first line treatment regimens are mainly centered on immunosuppressive therapy. This review assesses the role HRCT plays in optimizing care and improving clinical outcomes in SSc-ILD patients.

scholarly journals Three-month pulmonary function and radiological outcomes in COVID-19 survivors: a longitudinal patient cohort study

2020 ◽  
Author(s):  
Xuejiao Liao ◽  
Ying Wang ◽  
Ziyi He ◽  
Yongxing Yun ◽  
Ming Hu ◽  
...  
Keyword(s):  
Pulmonary Function ◽  
Hospital Discharge ◽  
Pulmonary Function Tests ◽  
Ground Glass Opacity ◽  
Ground Glass ◽  
High Resolution Computed Tomography ◽  
Airway Function ◽  
Function Test ◽  
Abnormal Pulmonary Function

Abstract Background This study aimed to investigate pulmonary function and radiological outcomes in a group of coronavirus disease 2019 (COVID-19) survivors. Methods 172 COVID-19 survivors in a follow-up clinic in a referral hospital underwent high resolution computed tomography (CT) of the thorax and pulmonary function tests at three month after hospital discharge. Results The median duration from hospital discharge to radiological and pulmonary function test was 90 (interquartile range=88-95) days. The abnormal pulmonary function was found in 11 (6.40%) patients, and abnormal small airway function (FEF25-75%) in 12 (6.98%). Six (3.49%) patients had obstructive ventilation impairment and six (3.49%) had restrictive ventilatory impairment. No significant differences in lung function parameters were observed between the non-severe and severe groups. Of 142 COVID-19 patients performed CT scan, 122 (85.91%) showed residual CT abnormalities and 52 (36.62%) showed chronic and fibrotic changes. The ground-glass opacities absorption in the lungs of severe cases was less satisfactory than that of non-severe patients. The severe paients had higher CT scores than non-severe cases (2.00 versus 0.00, P &lt; 0.001) Conclusion Of the COVID-19 survivors, 6.40% still present pulmonary function abnormality three month after discharge, which did not vary by disease severity during hospitalization. 85.91% patients had abnormalities on chest CT, with fibrous stripes and ground glass opacity as the most common pattern.

Interstitial lung disease in systemic sclerosis quantification of disease classification and progression with high-resolution computed tomography: An observational study

2021 ◽  
pp. 239719832098537
Author(s):  
Johan Clukers ◽  
Maarten Lanclus ◽  
Dennis Belmans ◽  
Cedric Van Holsbeke ◽  
Wilfried De Backer ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
Interstitial Lung Disease ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Severe Disease ◽  
Quantitative Computed Tomography ◽  
High Resolution Computed Tomography ◽  
Function Tests

Introduction: Systemic sclerosis–associated interstitial lung disease accounts for up to 20% of mortality in these patients and has a highly variable prognosis. Functional respiratory imaging, a quantitative computed tomography imaging technique which allows mapping of regional information, can provide a detailed view of lung structures. It thereby shows potential to better characterize this disease. Purpose: To evaluate the use of functional respiratory imaging quantitative computed tomography in systemic sclerosis–associated interstitial lung disease staging, as well as the relationship between short-term changes in pulmonary function tests and functional respiratory imaging quantitative computed tomography with respect to disease severity. Materials and methods: An observational cohort of 35 patients with systemic sclerosis was retrospectively studied by comparing serial pulmonary function tests and in- and expiratory high-resolution computed tomography over 1.5-year interval. After classification into moderate to severe lung disease and limited lung disease (using a hybrid method integrating quantitative computed tomography and pulmonary function tests), post hoc analysis was performed using mixed-effects models and estimated marginal means in terms of functional respiratory imaging parameters. Results: At follow-up, relative mean forced vital capacity percentage change was not significantly different in the limited (6.37%; N = 13; p = 0.053) and moderate to severe disease (−3.54%; N = 16; p = 0.102) groups, respectively. Specific airway resistance decreased from baseline for both groups. (Least square mean changes −25.11% predicted ( p = 0.006) and −14.02% predicted ( p = 0.001) for limited and moderate to severe diseases.) In contrast to limited disease from baseline, specific airway radius increased in moderate to severe disease by 8.57% predicted ( p = 0.011) with decline of lower lobe volumes of 2.97% predicted ( p = 0.031). Conclusion: Functional respiratory imaging is able to differentiate moderate to severe disease versus limited disease and to detect disease progression in systemic sclerosis.

scholarly journals Serum IGFBP-2 in Systemic Sclerosis as a Prognostic Factor of Lung Dysfunction

2021 ◽  
Author(s):  
Julien Guiot ◽  
Makon-Sébastien Njock ◽  
Béatrice André ◽  
Fanny Gester ◽  
Monique Henket ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Prognostic Factor ◽  
Pulmonary Function ◽  
Lung Disease ◽  
Pulmonary Function Tests ◽  
Serum Levels ◽  
Roc Curve Analysis ◽  
Lung Dysfunction ◽  
Patient’S Outcome

Abstract Background: Systemic sclerosis (SSc) is a rare connective tissue disease associated with rapid evolving interstitial lung disease (ILD), driving its mortality. Specific biomarkers associated with the progression of this lung disease are highly needed. We aimed to identify specific biomarkers of SSc-ILD to predict the evolution of the disease.Methods: We compared prospectively serum levels of several biomarkers associated with lung fibrosis in SSc patients (n=102), among which SSc-no ILD (n=63) and SSc-ILD (n=39), compared to healthy subjects (HS) (n=39). We also performed a longitudinal study in a subgroup of 28 patients analyzing biomarkers variations and pulmonary function tests over a period of 2 years.Results: Serum level of IGFBP-2 was significantly increased in SSc patients compared to HS, and negatively correlated with pulmonary function (assessed by carbon monoxide transfer coefficient (KCO)) (r=-0.29, p<0.01). Two-year longitudinal analysis in a subgroup of 28 SSc patients determined that IGFBP-2 variation was positively correlated with KCO at 2-year follow-up (r=0.6, p<0.001). SSc patients with a lower variation of IGFBP-2 (less than 22%) presented significant degradation of pulmonary function at 2-year follow-up (p<0.01). ROC curve analysis enabled us to identify that baseline IGFBP-2 > 105 ng/ml was associated with a poor outcome (KCO <70% predicted) at 2-year follow-up (AUC=0.75, p<0.05).Conclusions: We showed for the first time that serum levels of IGFBP-2 might be a prognostic factor of the development of SSc-ILD. Indeed, initial level of IGFBP-2 above 105 ng/ml was associated with a poor patient’s outcome (KCO <70% predicted) two years later.

scholarly journals AB0597 PULMONARY FUNCTION IN PATIENTS DIAGNOSED OF EARLY SYSTEMIC SCLEROSIS: A NEW TOOL FOR SYSTEMIC SCLEROSIS CLASIFFICATION?

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1595.1-1595
Author(s):  
F. M. Ortiz Sanjuan ◽  
C. Pávez Perales ◽  
E. Vicens Bernabeu ◽  
C. Alcañiz Escandell ◽  
I. Cánovas Olmos ◽  
...  
Keyword(s):  
Systemic Sclerosis ◽  
Pulmonary Function ◽  
Airway Obstruction ◽  
Pulmonary Involvement ◽  
Classification Criteria ◽  
Small Airway ◽  
University Hospital ◽  
Ventilatory Pattern ◽  
Small Airway Obstruction

Background:Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and is often progressive and has a poor prognosis. A restrictive ventilatory defect could suggest ILD either alone or in combination with pulmonary arterial hypertension.Nowadays, Early-SSc is well defined as preliminary stage of SSc. Patients who meet criteria for Early-SSc could benefit from an early diagnosis of pulmonary involvement.Objectives:Our aim was to assess the pulmonary function in patients diagnosed of Early SSc.Methods:Retrospective observational study of a wide and unselected series of patients diagnosed as Early-SSc from a single university hospital from 2012 to 2019. Patients were classified as Early-SSc following Le Roy criteria. Despite this, patients already did not meet 2013 ACR/EULAR classification criteria for SSc. We reviewed pulmonary function through conventional spirometry and diffusing capacity of lung for carbon monoxide (DLCO).Results:We included 56 patients with a mean age of 52.3±12.1 years (96.4% women; 3.6% men).At the diagnosis of Early-SSc, no one of our patients evidenced a restrictive ventilatory pattern. DLCO was below normal limits in 18 patients (32.1%). Small airway obstruction expressed according decreased maximal (mid-) expiratory flow (MMEF) 25-75 was present in 24 patients (42.8%).After a mean follow-up period of 38.3±2.4 months, 29 (51.8%) patients fulfilled 2013 ACR/EULAR criteria. The average time between diagnosis of Early-SSc and achieve SSc classification was 24.4±1.8 months. The remaining 27 patients continued classified as Early-SSc.An analysis of the subgroup of patients which progressed to SSc showed that DLCO was decreased in 15 of those 29 patients (51.7%) and 18 of 29 patients (62.1%) presented decreased MMEF 25-75. Comparing with the subgroup of patients which not progressed to SSc were significant differences (Decreased DLCO: 51.7% vs 11.1%; p=0.02 and decreased MMEF 25-75: 42.8% vs 22.2%; p=0.05).The analysis of pulmonary function of the subgroup of patients continued classified as Early-SSc after follow-up period did not show significative changes after follow-up.Conclusion:In our study, a third of the patients classified as Early-SSc presented at diagnosis abnormal values of DLCO and/or signs of small airway obstruction without the presence of a restrictive ventilatory pattern. Moreover, this pulmonary disfunction was significantly more frequent in patients who progressed to definitive SSc. Patients which remains classified as Early-SSc did not experience significative changes.Our results support the concept that pulmonary function was impaired in Early-SSc and that I should probably be considered for future Early-SSc classification criteria.Disclosure of Interests:None declared

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