Objective To investigate the lung involvement in Chinese patients with systemic sclerosis ( SSc) and its functional impact. Methods 68 patients with SSc were enrolled in Scleroderma study of PUMCH ( Peking Union Medical College Hospital) . All the patients underwent high resolution computed tomography ( HRCT) , pulmonary function testing, 6-minute walk testing, and echocardiography. And 36 patients filled in the Saint George’s Respiratory Questionnaire ( SGRQ) for assessment of healthrelated quality of life. Results HRCT revealed interstitial lung disease ( ILD) in 52 ( 76. 5% ) patients, 20 out of them without respiratory symptoms. Reticular opacification, ground-glass opacity ( GGO) , traction bronchiectasis, and honeycomb were presented respectively in 80.8% , 73.1% , 59.6% , and 30.8% of patients with SSc-ILD. Fibrosis was the predominant HRCT finding, and pure GGO ( in the absence of reticulation or architectural distortion) was only present in 8 ( 15. 7% ) patients. 57 (83.8% ) patients presented with diffusion defect, with most of them having moderate to severe impairent. Reduced FVC or TLC presented in 20 ( 29.4% ) and 28 ( 41.2% ) of patients respectively. The significant correlation was observed between the DLCO and the extent of ILD on HRCT ( rs = -0.476, P =0.000) . DLCO showed significant correlations with all the four components of the SGRQ ( Plt;0.05) . Significant correlations were also observed among the SGRQ scores and SpO2 ( maximum desaturation) or Borg index. Stepwise multiple regression analysis confirmed that the DLCO, SpO2 , and Borg index contributed to the SGRQ. Conclusions Lung involvement in Chinese SSc patients is common and insidious. The HRCT features of SSc-ILD are predominant fibrosis plus GGO, indicating little reversibility. Thus HRCT should be performed routinely in newly diagnosed SSc patients for early screening of lung involvement. The lung function defect of SSc is characterized by reduced diffusing capacity, and DLCO show better correlations with HRCT abnormities or SGRQ than FVC or TLC. Thus DLCO is of great value for early detection or severity assessment of SSc-ILD. SGRQ can be used to examine the health related quality of life of SSc patients and may reflect severity of lung involvement.
Objective To systematically review whether the prevalence of left ventricular diastolic dysfunction was higher in systemic sclerosis (SSc) patients. Methods The Cochrane Library, PubMed, EMbase, CBM, CNKI and WanFang Data databases were electronically searched to collect the studies about comparing echocardiographic parameters in SSc patients and controls from January 1990 to June 2016. Two reviewers independently screened literature, extracted data and assessed the risk of bias of included studies, then, meta-analysis was performed by using RevMan 5.3 software. Results A total of 22 studies involving 1 146 patients were included. The results of meta-analysis showed that: compared to controls, patients with SSc had prolonged left isovolumetric relaxation time (MD=10.40, 95%CI 4.04 to 16.77, P=0.001), higher trans-mitral A-wave velocity (MD=0.11, 95%CI 0.07 to 0.15, P<0.000 01), prolonged mitral deceleration time (MD=8.04, 95%CI 2.66 to 13.42,P=0.003), larger mean left atrial dimension (MD=1.43, 95%CI 0.11 to 2.76, P=0.03), higher estimated pulmonary artery pressure (MD=11.35, 95%CI 6.08 to 16.6, P<0.001), higher E/E’ ratio (MD=2.08, 95%CI 0.19 to 3.96,P=0.03) and lower trans-mitral E-wave velocity (MD=–0.03, 95%CI –0.05 to –0.01, P=0.000 3), mitral E/A ratio (MD=–0.24, 95%CI –0.32 to –0.15, P<0.000 01) and trans-mitral E’-wave velocity (MD=–1.52, 95%CI –2.44 to –0.60,P=0.001). There were no differences in left ventricular ejection fraction, isovolumetric end-systolic dimension, septal end-diastolic thickness and posterior wall end-diastolic thickness, trans-mitral A’-wave velocity, E’/A’ ratio. Conclusion SSc patients are more likely to have echocardiographic parameters of LVDD. Due to limited quality and quantity of the included studies, more high quality studies are needed to verify above conclusion.
Objective To analyze the pathways, biomarkers and diagnostic genes of systemic sclerosis associated interstitial lung disease (SSc-ILD) using bioinformatics. Methods SSc-ILD related gene data sets from April to June 2023 were downloaded from the Gene Expression Omnibus database for differential analysis and enrichment analyses including gene ontology analysis, Kyoto Encyclopedia of Genes and Genomes analysis, disease ontology analysis, and gene set enrichment analysis. Least absolute shrinkage and selection operator regression and support vector machine algorithms were applied to screen and take the intersection to get the diagnostic genes and validate the results. Disease-related data were analyzed by immune cell infiltration. Results A total of 178 differential genes were obtained, and enrichment analyses showed that they were related to 5 signaling pathways and associated with 3 diseases. The diagnostic genes screened were TNFAIP3, ID3, and NT5DC2, and immune cell infiltration showed that the diagnostic genes were associated with plasma cells, resting mast cells, activated natural killer cells, macrophage M1 and M2, resting dendritic cells, and activated dendritic cells. Conclusion The screened diagnostic genes and immune cells may be involved in the development of SSc-ILD.