scholarly journals Prediction of long-term mortality by using machine learning models in Chinese patients with connective tissue disease-associated interstitial lung disease

2022 ◽  
Vol 23 (1) ◽  
Author(s):  
Di Sun ◽  
Yu Wang ◽  
Qing Liu ◽  
Tingting Wang ◽  
Pengfei Li ◽  
...  
Keyword(s):  
Machine Learning ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Mortality Risk ◽  
Curve Analysis ◽  
Gap Model ◽  
Decision Curve Analysis ◽  
Likelihood Ratio Testing

Abstract Background The exact risk assessment is crucial for the management of connective tissue disease-associated interstitial lung disease (CTD-ILD) patients. In the present study, we develop a nomogram to predict 3‑ and 5-year mortality by using machine learning approach and test the ILD-GAP model in Chinese CTD-ILD patients. Methods CTD-ILD patients who were diagnosed and treated at the First Affiliated Hospital of Zhengzhou University were enrolled based on a prior well-designed criterion between February 2011 and July 2018. Cox regression with the least absolute shrinkage and selection operator (LASSO) was used to screen out the predictors and generate a nomogram. Internal validation was performed using bootstrap resampling. Then, the nomogram and ILD-GAP model were assessed via likelihood ratio testing, Harrell’s C index, integrated discrimination improvement (IDI), the net reclassification improvement (NRI) and decision curve analysis. Results A total of 675 consecutive CTD-ILD patients were enrolled in this study, during the median follow-up period of 50 (interquartile range, 38–65) months, 158 patients died (mortality rate 23.4%). After feature selection, 9 variables were identified: age, rheumatoid arthritis, lung diffusing capacity for carbon monoxide, right ventricular diameter, right atrial area, honeycombing, immunosuppressive agents, aspartate transaminase and albumin. A predictive nomogram was generated by integrating these variables, which provided better mortality estimates than ILD-GAP model based on the likelihood ratio testing, Harrell’s C index (0.767 and 0.652 respectively) and calibration plots. Application of the nomogram resulted in an improved IDI (3- and 5-year, 0.137 and 0.136 respectively) and NRI (3- and 5-year, 0.294 and 0.325 respectively) compared with ILD-GAP model. In addition, the nomogram was more clinically useful revealed by decision curve analysis. Conclusions The results from our study prove that the ILD-GAP model may exhibit an inapplicable role in predicting mortality risk in Chinese CTD-ILD patients. The nomogram we developed performed well in predicting 3‑ and 5-year mortality risk of Chinese CTD-ILD patients, but further studies and external validation will be required to determine the clinical usefulness of the nomogram.

scholarly journals POS1419 PREDICTIVE FACTORS FOR PULMONARY PROGRESSION IN PATIENTS WITH CONNECTIVE TISSUE DISEASE AND INTERSTITIAL LUNG DISEASE

2021 ◽  
Vol 80 (Suppl 1) ◽  
pp. 993.1-993
Author(s):  
Y. H. Chiu ◽  
J. Spierings ◽  
P. De Jong ◽  
F. Mohamed Hoesein ◽  
J. M. Van Laar ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Mortality Risk ◽  
Cox Regression ◽  
High Resolution Computed Tomography ◽  
Kaplan Meier ◽  
Risk Of Mortality

Background:Interstitial lung disease (ILD) is associated with decreased quality of life and higher mortality risk in patients with connective tissue disease (CTD). Outcome and treatment response to immunosuppressive therapies is unpredictable, and therefore the management of CTD-ILD can be challenging.Objectives:Our study aimed to identify clinical and imaging factors that are predictive for outcome in patients with CTD-ILD.Methods:We performed a retrospective cohort study in patients with CTD-ILD who were treated in our centre between 2004 and 2018. Clinical, biochemical data as well as pulmonary function test (PFT) and high-resolution computed tomography (HRCT) results were recorded. Two experienced chest radiologists independently and blindly reviewed the HRCT’s. When the two chest radiologists assessed the ILD pattern differently, a diagnosis was made by consultation of a third expert. The ILD patterns were classified as fibrotic or inflammatory. Overall survival and progressive fibrosing interstitial lung disease (PF-ILD, defined as a significant decline of PFT and HRCT) after two years of treatment were assessed using a Kaplan-Meier plot. Multivariable Cox regression was including for treatment, comorbidity, and age as variables. Factors with a p value < 0.2 in the univariate analysis were included in the multivariate analysis. The correlation between the variation of serum markers and PFT over-time was evaluated with Spearman’s Rho.Results:In total, 150 patients with CTD-ILD were included, of which 53 (35.3%) had systemic sclerosis, 19 (12.7%) Sjogren’s syndrome, 29 (19.3%) inflammatory myopathy, 24 (16%) rheumatoid arthritis, 5 (3.3%) systemic lupus erythematosus, 4 (2.7%) mixed connective tissue disease, and 16 (10.7%) undifferentiated connective tissue disease patients. Median disease duration of CTD was 14 months (IQR 2–73) in patients with CTD diagnosis before ILD onset. The median follow-up duration was 40 months (IQR 27.3–60.8). Thirty (20%) deaths occurred, in which the cause of death was a pulmonary infection in 6 (4%) patients and a respiratory failure due to ILD in 10 (6.7%) patients. PF-ILD occurred in 82 (54.7%) patients, which was associated with poor overall survival (HR 3.03, 95%CI 1.15–7.98) (Figure 1). Age, smoking, and steroid usage were associated with increased mortality risk as well (Table 1). There was no dose-related effect of smoking on mortality.Figure 1.The Kaplan-Meier plot for progressive fibrosing interstitial lung diseases (PF-ILD). PF-ILF was defined as pulmonary function decline or high-resolution computed tomography progression after two years of treatment.Inflammatory patterns on baseline HRCT were correlated with a lower risk of FVC decline than fibrotic patterns (OR 0.24, 95%CI 0.09–0.64). The increase in CA15.3 level was associated with the decline in FVC (Rho -0.308, p=0.037). Besides, the elevation in CRP was associated with the reduction in FVC (Rho -0.302, p=0.006) and DLCO (Rho -0.268, p=0.019).Conclusion:Our study identified several factors associated with outcomes. Age, smoking, and steroid treatment increased the risk of mortality in patient with CTD-ILD. Inflammatory HRCT pattern at baseline revealed a better pulmonary outcome than a fibrotic pattern. The patients having PF-ILD after two years of treatment showed a higher mortality risk.Table 1.Multivariable Cox-regression for the clinical risk of mortality.Clinical factorCrude HR (95%CI)PAdjusted HR (95%CI)pAge1.11 (1.06–1.15)1.7*10-61.12 (1.07–1.17)3.54*10-6Smoking1.64 (0.79–3.43)0.1872.53 (1.11–5.78)0.028Congestive heart failure1.86 (0.75–4.58)0.1791.17 (0.47–2.91)0.737MMF0.55 (0.23–1.35)0.1950.73 (0.29–1.85)0.512Steroid4.37 (1.67–11.45)0.0034.96 (1.84–13.40)0.002MMF, mycophenolate mofetil; HR, hazard ratio.Acknowledgements:We want to thank Marieke Vianen for the support in data management, Lieke Wintermans and Lisa Hessels for collecting the clinical data.Disclosure of Interests:None declared

scholarly journals Lung cancer development in patients with connective tissue disease–related interstitial lung disease

Medicine ◽  
2016 ◽  
Vol 95 (50) ◽  
pp. e5716 ◽  
Author(s):  
Yasunori Enomoto ◽  
Naoki Inui ◽  
Katsuhiro Yoshimura ◽  
Koji Nishimoto ◽  
Kazutaka Mori ◽  
...  
Keyword(s):  
Lung Cancer ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Cancer Development

scholarly journals Treatment of Pulmonary Hypertension in Patients with Connective Tissue Disease and Interstitial Lung Disease

2010 ◽  
Vol 17 (6) ◽  
pp. 282-286 ◽  
Author(s):  
Shikha Mittoo ◽  
Thomas Jacob ◽  
Andrea Craig ◽  
Zoheir Bshouty
Keyword(s):  
Pulmonary Hypertension ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Term Treatment ◽  
Kaplan Meier ◽  
Long Term Treatment ◽  
Survival Differences

BACKGROUND: Pulmonary hypertension (PH) in patients with connective tissue disease (CTD) can occur in isolation or concomitantly with interstitial lung disease (ILD). Targeted therapies for PH can mitigate clinical deterioration in CTD patients with isolated PH; however, the effect of these therapies in CTD patients with PH and ILD (CTD-PH-ILD) are poorly characterized.OBJECTIVE: To investigate outcomes following long-term treatment of PH in patients with CTD-PH-ILD.METHODS: A retrospective evaluation of 13 CTD-PH-ILD patients who were treated with bosentan, sildenafil or bosentan plus sildenafil, was conducted. Immunosuppressants were prescribed as indicated. Patients underwent pulmonary function testing and assessment of 6 min walk distance at the time of treatment initiation and during follow-up. Patients were followed until time of death, lung transplantation or the end of the study. Kaplan-Meier estimates of survival were calculated and log-rank testing was used to analyze survival differences according to CTD subtype.RESULTS: Thirteen patients (seven with systemic sclerosis [SSc], four with overlap syndrome, and two with rheumatoid arthritis) were followed for a mean (± SD) duration of 33.8±21.7 months. The survival estimate at a median duration of 34 months was 85%; two patients with SSc died. Mortality rates were greater among patients with SSc versus other CTD subtypes (P=0.04). No changes from baseline to follow-up in mean forced vital capacity or exercise capacity, and no treatment-related toxicity, were observed.CONCLUSION: Treatment using PH-specific therapies in patients with CTD, PH and ILD was well tolerated. Further studies to investigate the efficacy of PH-specific therapies in CTD-PH-ILD patients are warranted.

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