A novel CT scoring method predicts the prognosis of interstitial lung disease associated with anti-MDA5 positive dermatomyositis

PurposeThe purpose of this study is to analyze clinical information and combine significant parameters to generate a predictive model and achieve a better prognosis prediction of dermatomyositis-associated interstitial lung disease with positive melanoma differentiation-associated gene 5 antibody (MDA5+ DM-ILD) and stratify patients according to prognostic risk factors appropriately.MethodsWe retrospectively reviewed 63 patients MDA5+ DM-ILD who were treated in our hospital from January 2018 to January 2021. Our study incorporated most clinical characteristics in clinical practice to explore the associations and predictive functions of clinical characteristics and prognosis. Student's t-test, Mann-Whitney U-test, chi-squared test, Pearson correlation analysis, Cox regression analysis, R, receiver operating characteristic curves (ROC curves), and Kaplan-Meier survival curves were performed to identify independent predictors for the prognosis of MDA5+DM-ILD.ResultsIn all the 63 patients with MDA5+DM-ILD, 44 improved but 19 did not. Poor prognosis was found more frequently in patients who were older, clinically amyopathic variant of dermatomyositis (CADM), and/or with short duration, short interval of DM and ILD, long length of stay, fever, dyspnea, non-arthralgia, pulmonary infection, pleural effusion (PE), high total computed tomography scores (TCTs), ground-glass opacity (GGO), consolidation score, reticular score and fibrosis score, decreased forced vital capacity (FVC), forced expiratory volume in 1s (FEV1), albumin, A/G, glomerular filtration rate (GFR) and tumor necrosis factor α (TNFα), high titer of anti-MDA5, proteinuria, high levels of monocyte, lactate dehydrogenase (LDH), ferritin (FER), neuron specific enolase (NSE) and glucocorticoid, antibiotic, antiviral, and non-invasive positive pressure ventilation (NPPV). The multivariate Cox regression analysis demonstrated that duration, fever, PE, TCTs and aspartate transaminase (AST) were independent predictors of poor prognosis in patients with MDA5+DM-ILD. The nomogram model quantified the risk of 400-day death as: duration ≤ 4 months (5 points), fever (88 points), PE (21 points), TCTs ≥10 points (22 points), and AST ≥200 U/L (100 points) with high predictive accuracy and convenience. The ROC curves possessed good discriminative ability for combination of fever, PE, TCTs, and AST, as reflected by the area under curve (AUC) being.954, 95% CI 0.902–1.000, and sensitivity and specificity being 84.2 and 94.6%, respectively.ConclusionWe demonstrated that duration, fever, PE, TCTs, and AST could be integrated together to be independent predictors of poor prognosis in MDA5+ DM-ILD with highly predictive accuracy.

Section: Resultsmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

A New Predictive Model for the Prognosis of MDA5+ DM-ILD

Niu

Zhao

et al. 2022

“…However, currently available HRCT scoring methods are observer dependent, with considerable inter-observer bias and initially derived for idiopathic pulmonary fibrosis (IPF) (8)(9)(10). When referring to MDA5 + DM-ILD with a much more aggressive clinical course, the applicability of the scoring system is controversial (11). Therefore, a measurable and quantitative method to assess severity and predict prognosis of MDA5 + DM-ILD is urgently needed.…”

Section: Introductionmentioning

confidence: 99%

A Computed Tomography Radiomics-Based Prediction Model on Interstitial Lung Disease in Anti-MDA5-Positive Dermatomyositis

Zheng³

et al. 2021

Self Cite

Objectives: Anti-melanoma differentiation-associated gene 5-positive dermatomyositis-associated interstitial lung disease (MDA5+ DM-ILD) is a life-threatening disease. The current study aimed to quantitatively assess the pulmonary high-resolution computed tomography (HRCT) images of MDA5+ DM-ILD by applying the radiomics approach and establish a multidimensional risk prediction model for the 6-month mortality.Methods: This retrospective study was conducted in 228 patients from two centers, namely, a derivation cohort and a longitudinal internal validation cohort in Renji Hospital, as well as an external validation cohort in Guangzhou. The derivation cohort was randomly divided into training and testing sets. The primary outcome was 6-month all-cause mortality since the time of admission. Baseline pulmonary HRCT images were quantitatively analyzed by radiomics approach, and a radiomic score (Rad-score) was generated. Clinical predictors selected by univariable Cox regression were further incorporated with the Rad-score, to enhance the prediction performance of the final model (Rad-score plus model). In parallel, an idiopathic pulmonary fibrosis (IPF)-based visual CT score and ILD-GAP score were calculated as comparators.Results: The Rad-score was significantly associated with the 6-month mortality, outperformed the traditional visual score and ILD-GAP score. The Rad-score plus model was successfully developed to predict the 6-month mortality, with C-index values of 0.88 [95% confidence interval (CI), 0.79–0.96] in the training set (n = 121), 0.88 (95%CI, 0.71–1.0) in the testing set (n = 31), 0.83 (95%CI, 0.68–0.98) in the internal validation cohort (n = 44), and 0.84 (95%CI, 0.64–1.0) in the external validation cohort (n = 32).Conclusions: The radiomic feature was an independent and reliable prognostic predictor for MDA5+ DM-ILD.

“…A recent study used deep learning and feature learning to obtain better segmentation results by training with large samples (23). Software package named "CT Pneumonia Analysis" applying in the similar mechanism was used in study by S Ye recently (24). Our design idea was to extent different.…”

Section: Discussionmentioning

confidence: 99%

The Value of Effective Lung Ventilation Area Ratio Based on CT Image Analysis Is a New Index to Predict the Shorter Outcome of Anti-melanoma Differentiation-Associated Protein 5 Positive Dermatomyositis Associated Interstitial Lung Disease: A Single-Center Retrospective Study

Wang

Mei

et al. 2021

Background: Anti-melanoma differentiation-associated protein 5 (MDA5) positive dermatomyositis (MDA5+DM) patients have poor outcomes due to rapidly progressive interstitial lung disease (ILD). The accurate assessment of lung involvement is an urgent focus of research.Methods: A computer-aided lung interstitial image analysis technology has been developed, and a quantitative indicator named effective lung ventilation area ratio (ELVAR) that calculates the proportion of the area outside the lung interstitium in lung tissue has been established. 55 newly diagnosed MDA5+DM patients and 46 healthy individuals, matched for age and gender, were enrolled in this study. MDA5+DM patients were classified into early death group or early survival group according to their survival state within 3 months after diagnosis. Clinical characteristics, laboratory and immunological test results, lung involvement (including ELVAR value) and treatment were compared between early death group and early survival group to determine an index that can predict prognoses of patients with MDA5+DM.Results: There were significant differences between early death MDA5+DM patients and early survival MDA5+DM patients about 12 indices including age of onset, CRP, ferritin, albumin, and pulmonary involvement including severity of type I respiratory failure at diagnosis, P/F ratio, oxygen supplementation, values of ELVAR, FVC, and DLCO. The results of ROC analysis and correlation analysis showed the value of ELVAR had good diagnostic value and widely correlation with many clinical characteristics. Univariate analysis and Multivariate analysis showed four factors including age of onset, ferritin, value of ELVAR, and oxygen supplementation >4 L/min significantly value for poor prognosis in MDA5+DM patients. A cutoff value of 0.835 about ELVAR had good predictive power for mortality within 3 months in 54.2% of MDA5+DM patients.Conclusion: The value of ELVAR derived from computed tomography image analysis is a new index that can predict poor outcomes in MDA5+DM patients with rapidly progressive interstitial lung disease.