Cardiopulmonary exercise testing and prognosis in patients with systemic sclerosis without baseline pulmonary hypertension: a prospective cohort study

Bournia, Vasiliki-Kalliopi; Kallianos, Anastasios; Panopoulos, Stylianos; Gialafos, Elias; Velentza, Lemonia; Vlachoyiannopoulos, Panayiotis G.; Sfikakis, Petros P.; Trakada, Georgia

doi:10.1007/s00296-021-04937-w

Cited by 5 publications

(3 citation statements)

References 36 publications

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“…Surprisingly, we found that the 6MWT results did not differ between the SSc-ILD and IPF patients, although the SSc-ILD patients were significantly younger and therefore were expected to have better functional performance compared to older IPF patients. The underlying mechanisms might have included vascular manifestations such as PH and musculoskeletal conditions affecting exercise limitation [ 25 , 26 , 27 ].…”

Section: Discussionmentioning

confidence: 99%

Clinical Predictors of Lung-Function Decline in Systemic-Sclerosis-Associated Interstitial Lung Disease Patients with Normal Spirometry

et al. 2022

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Interstitial lung disease (ILD) is the leading cause of mortality in systemic sclerosis (SSc). Progressive pulmonary fibrosis (PPF) is defined as progression in 2 domains including clinical, radiological or lung-function parameters. Our aim was to assess predictors of functional decline in SSc-ILD patients and compare disease behavior to that in idiopathic pulmonary fibrosis (IPF) patients. Patients with normal forced vital capacity (FVC > 80% predicted; SSc-ILD: n = 31; IPF: n = 53) were followed for at least 1 year. Predictors of functional decline including clinical symptoms, comorbidities, lung-function values, high-resolution CT pattern, and treatment data were analyzed. SSc-ILD patents were significantly younger (59.8 ± 13.1) and more often women (93 %) than IPF patients. The median yearly FVC decline was similar in both groups (SSc-ILD = −67.5 and IPF = −65.3 mL/year). A total of 11 SSc-ILD patients met the PPF criteria for functional deterioration, presenting an FVC decline of −153.9 mL/year. Cough and pulmonary hypertension were significant prognostic factors for SSc-ILD functional progression. SSc-ILD patients with normal initial spirometry presenting with cough and PH are at higher risk for showing progressive functional decline.

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Section: Discussionmentioning

confidence: 99%

Clinical Predictors of Lung-Function Decline in Systemic-Sclerosis-Associated Interstitial Lung Disease Patients with Normal Spirometry

et al. 2022

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“… 22 In the absence of baseline PAH, CPET indices may predict pulmonary function deterioration and death in SSc patients. 23 However, this was not the case in our study because we did not undertake it. Third, considering the potential side effects of contrast medium administration, postcontrast CMR imaging was not performed in this study.…”

Section: Discussionmentioning

confidence: 79%

Diffusing capacity of lungs for carbon monoxide associated with subclinical myocardial impairment in systemic sclerosis: A cardiac MR study

He,

Tong,

Ning

et al. 2023

RMD Open

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BackgroundSystemic sclerosis (SSc) is characterised by microvascular and fibrotic lesions, which are located not only in skin but also in lungs and heart.ObjectiveThis study aimed to investigate the association between lung function and myocardial T1 values using cardiac MR (CMR) imaging in patients with SSc without cardiovascular symptoms.MethodsThe SSc patients and age- and sex-matched healthy subjects underwent CMR. The cardiac function and native T1 values of myocardium and lung function were measured. Spearman’s rank correlations and linear regression analyses were performed to determine the association between lung function and myocardial T1.ResultsForty-five SSc patients (aged 47.7±13.2 years, 40 females) and 23 (aged 46.0±14.4 years, 20 females) healthy subjects were enrolled. SSc patients exhibited considerably higher native T1 values compared with healthy subjects (1305.9±49.8 ms vs 1272.6±37.6 ms, p=0.006). Linear regression analysis revealed that decrease of diffusing capacity of lungs for carbon monoxide (DLCO) in SSc patients was notably associated with myocardial native T1 value before (β –1.017; 95% CI –1.883 to –0.151; p=0.022) and after adjusting for confounding factors (β –1.108; 95% CI −2.053 to –0.164; p=0.023). Moderate-to-severe decrease of DLCO was found to be significantly associated with myocardial native T1 value (β 48.006; 95% CI 17.822 to 78.190; p=0.003) after adjusting for confounding factors.ConclusionDLCO inversely correlates with myocardial native T1 values in SSc patients, particularly moderate-to-severely decreased DLCO, suggesting that DLCO might be a potential indicator for subclinical myocardial impairment in SSc patients.

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“…PFT can reflect the information of various physiological and pathological changes of the lung, including the ventilation function of the airway and the gas exchange function of the vascular interstitium [ 18 ]. The relationship between group I of PH and parameters related to pulmonary ventilation function is still controversial, but the decline of DLCO, a parameter reflecting gas exchange function, is considered to be a characteristic change of pulmonary function in most patients with PAH [ 27 , 28 ]. The decline in DLCO is the comprehensive result of endothelial cell proliferation leading to thickening of alveolar capillary membranes, increased PVR leading to decreased pulmonary vascular blood volume, decreased right ventricular output, and local thrombosis [ 29 ].…”

Section: Discussionmentioning

confidence: 99%

The Role of Pulmonary Function Test for Pulmonary Arterial Hypertension in Patients with Connective Tissue Disease

Xiong

Huang

et al. 2022

Disease Markers

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Objective: The study aimed to investigate the value of pulmonary function test (PFT) in evaluating and predicting pulmonary arterial hypertension (PAH) in patients with connective tissue disease (CTD). Methods: This was a prospective observational study recruiting patients diagnosed with CTD-PAH. Patients with interstitial lung disease and pulmonary hypertension induced by other causes were not eligible for enrollment. All patients were assessed for PAH every 1–3 months. A patient was considered to have clinical improvement if the grade of risk stratification declined or at least two parameters improved during follow-up, otherwise no improvement. Results: A total of 31 patients with CTD-PAH were recruited in this study. Nearly 70% of patients had declined forced vital capacity (FVC), 60% had declined total lung capacity and maximum expiratory flow at 50% of vital capacity, and 95% had normal or mild decline in forced expiratory volume in 1 second (FEV1)/FVC. A decline in diffusing capacity of the lung for carbon monoxide (DLCO) was present in 96% of patients, and 60% were moderate to severe. Furthermore, 50% of patients had an FVC/DLCO ratio of less than 1.4. Univariate analysis showed that FEV1/FVC, DLCO, and FVC/DLCO were associated with disease prognosis. After adjusting for age as a confounding factor, multivariate logistic regression analysis revealed that DLCO was an independent predictive factor for the prognosis of CTD-PAH. Conclusion: The pulmonary function of patients with CTD-PAH is abnormal in parameters such as lung volume, small airway, and gas exchange. PFT can reveal complex pathophysiological changes in the lungs of CTD-PAH patients and predict prognosis.

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Cardiopulmonary exercise testing and prognosis in patients with systemic sclerosis without baseline pulmonary hypertension: a prospective cohort study

Cited by 5 publications

References 36 publications

Clinical Predictors of Lung-Function Decline in Systemic-Sclerosis-Associated Interstitial Lung Disease Patients with Normal Spirometry

Clinical Predictors of Lung-Function Decline in Systemic-Sclerosis-Associated Interstitial Lung Disease Patients with Normal Spirometry

Diffusing capacity of lungs for carbon monoxide associated with subclinical myocardial impairment in systemic sclerosis: A cardiac MR study

The Role of Pulmonary Function Test for Pulmonary Arterial Hypertension in Patients with Connective Tissue Disease

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