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 AB0605 CLINICAL PROFILE AND CHEST HIGH-RESOLUTION COMPUTED TOMOGRAPHY (HRCT) FINDINGS IN PATIENTS WITH CONNECTIVE TISSUE DISEASES AND INTERSTITIAL LUNG DISEASE: EXPERIENCE OF A SINGLE REFERENCE RHEUMATOLOGY CENTER

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1598.2-1599
Author(s):  
I. Rusu ◽  
L. Muntean ◽  
M. M. Tamas ◽  
I. Felea ◽  
L. Damian ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Systemic Sclerosis ◽  
High Resolution ◽  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Interstitial Pneumonia ◽  
Connective Tissue Diseases ◽  
High Resolution Computed Tomography ◽  
Hrct Patterns

Background:Interstitial lung disease (ILD) is a common manifestation of connective tissue diseases (CTDs), and is associated with significant morbidity and mortality. Chest high-resolution computed tomography (HRCT) play an important role in the diagnosis of ILD and may provide prognostic information.Objectives:We aimed to characterize the clinical profile and chest HRCT abnormalities and patterns of patients diagnosed with CTDs and ILD.Methods:In this retrospective, observational study we included 80 consecutive patients with CTDs and ILD referred to a tertiary rheumatology center between 2015 and 2019. From hospital charts we collected clinical data, immunologic profile, chest HRCT findings. HRCT patterns were defined according to new international recommendations.Results:Out of 80 patients, 64 (80%) were women, with a mean age of 55 years old. The most common CTD associated with ILD was systemic sclerosis (38.8%), followed by polymyositis (22.5%) and rheumatoid arthritis (18.8%). The majority of patients had dyspnea on exertion (71.3%), bibasilar inspiratory crackles were present in 56.3% patients and 10% had clubbing fingers. Antinuclear antibodies (ANA) were present in 78.8% patients, and the most frequently detected autoantibodies against extractable nuclear antigen were anti-Scl 70 (28.8%), followed by anti-SSA (anti-Ro, 17.5%), anti-Ro52 (11.3%) and anti-Jo (7.5%). Intravenous cyclophosphamide therapy for 6-12 months was used in 35% of patients, while 5% of patients were treated with mycophenolate mofetil.The most frequent HRCT abnormalities were reticular abnormalities and ground glass opacity. Non-specific interstitial pneumonia (NSIP) was identified in 46.3% CTDs patients. A pattern suggestive of usual interstitial pneumonia (UIP) was present in 32.5% patients, mainly in patients with systemic sclerosis. In 21.3% patients the HRCT showed reticulo-nodular pattern, micronodules and other abnormalities, not diagnostic for UIP or NSIP pattern.Conclusion:Nonspecific interstitial pneumonia (NSIP) is the most common HRCT pattern associated with CTDs. Further prospective longitudinal studies are needed in order to determine the clinical and prognostic significance of various HRCT patterns encountered in CTD-associated ILD and for better patient management.References:[1]Ohno Y, Koyama H, Yoshikaua T, Seki S. State-of-the-Art Imaging of the Lung for Connective Tissue Disease (CTD). Curr Rheumatol Rep. 2015;17(12):69.[2]Walsh SLF, Devaraj A, Enghelmeyer JI, Kishi K, Silva RS, Patel N, et al. Role of imaging in progressive-fibrosing interstitial lung diseases. Eur Respir Rev. 2018;27(150)Disclosure of Interests:None declared

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.

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 The value of serum Krebs von den lungen-6 as a diagnostic marker in connective tissue disease associated with interstitial lung disease

2020 ◽  
Vol 20 (1) ◽  
Author(s):  
Hua Ma ◽  
Junhui Lu ◽  
Yuanyuan Song ◽  
Huixuan Wang ◽  
Songlou Yin
Keyword(s):  
Interstitial Lung Disease ◽  
Connective Tissue ◽  
Lung Disease ◽  
Connective Tissue Disease ◽  
Diagnostic Marker ◽  
Pulse Therapy ◽  
Enzyme Linked Immunosorbent Assay ◽  
High Resolution Computed Tomography ◽  
The Difference ◽  
Diagnostic Indicator

Abstract Objectives The purpose of this study was to evaluate the value of serum krebs von den lungen-6 (KL-6) level as a diagnostic indicator for connective tissue disease associated with interstitial lung disease (CTD-ILD). Methods One hundred fifty five patients with newly diagnosed CTD in our hospital were enrolled and divided into two groups by their ILD manifestations, the CTD-ILD group and the CTD group. In parallel, 61 patients with pulmonary infection and 60 cases of healthy subjects were also enrolled into the study. The difference of serum KL-6 level among the four groups were compared. In CTD-ILD group, carbon monoxide diffusing capacity (DLCo) and high-resolution computed tomography (HRCT) of lung were also tested. The serum KL-6 level of 32 patients from the CTD-ILD group who received cyclophosphamide (CTX) pulse therapy were sampled and measured, by enzyme linked immunosorbent assay (ELISA), at three time points: before treatment, 3 months after treatment and 6 months after treatment. Results The serum KL-6 level in the CTD-ILD group (1004.9 (676.41738.1) IU/ml) is significantly higher than three other groups (χ2 = 72.29, P < 0.001). In the CTD-ILD group the level of serum KL-6 was positively correlated with disease severity on HRCT (r = 0.75, P <  0.001), while was negatively correlated with DLCo (r = − 0.50, P <  0.001). In 32 patients who received CTX pulse therapy, the level of serum KL-6 was gradually decreased in 20 cases whose lesions were absorbed within 6 months (F = 13.67, P <  0.001), whereas it remained unchanged in the rest of 12 patients (Z = -1.328, P = 0.198). Conclusions Serum KL-6 level can potentially serve as a diagnostic marker for CTD-ILD and be utilized to evaluate the effectiveness of CTX pulse therapy.

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

2021 ◽  
Vol 8 ◽  
Author(s):  
Wenwen Xu ◽  
Wanlong Wu ◽  
Yu Zheng ◽  
Zhiwei Chen ◽  
Xinwei Tao ◽  
...  
Keyword(s):  
Computed Tomography ◽  
Interstitial Lung Disease ◽  
Prediction Model ◽  
Lung Disease ◽  
Cox Regression ◽  
Validation Cohort ◽  
External Validation ◽  
High Resolution Computed Tomography ◽  
Internal Validation ◽  
Derivation Cohort

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.

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