scholarly journals 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

2021 ◽  
Vol 8 ◽  
Author(s):  
Changjian Wang ◽  
Jinfeng Du ◽  
Xilong Mei ◽  
Lingchao Guo ◽  
Fangzhao Li ◽  
...  
Keyword(s):  
Image Analysis ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Age Of Onset ◽  
Lung Ventilation ◽  
Early Death ◽  
Survival Group ◽  
Early Survival ◽  
Poor Outcomes ◽  
Death Group

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.

scholarly journals The comparison of nailfold videocapillaroscopy findings between anti-melanoma differentiation-associated gene 5 antibody and anti-aminoacyl tRNA synthetase antibody in patients with dermatomyositis complicated by interstitial lung disease

2020 ◽  
Vol 10 (1) ◽  
Author(s):  
Reiko Wakura ◽  
Shogo Matsuda ◽  
Takuya Kotani ◽  
Takeshi Shoda ◽  
Tohru Takeuchi
Keyword(s):  
Interstitial Lung Disease ◽  
Disease Activity ◽  
Lung Disease ◽  
Trna Synthetase ◽  
Aminoacyl Trna Synthetase ◽  
Nailfold Videocapillaroscopy ◽  
High Resolution Computed Tomography ◽  
Survival Group ◽  
Positive Group ◽  
Death Group

Abstract Dermatomyositis (DM) is frequently complicated by interstitial lung disease (ILD), which increases mortality. This study aims to elucidate the clinical significance of nailfold videocapillaroscopy (NVC) on assessing the disease activity and prognosis of DM-ILD. We compared the NVC findings between anti-melanoma differentiation-associated gene 5 (anti-MDA5) antibody-positive and anti-aminoacyl tRNA synthetase (anti-ARS) antibody-positive patients, the survival and ILD-related death groups, and examined the association of NVC findings with prognostic factors of DM-ILD. The median scores of microhemorrhage and capillary disorganization in the anti-MDA5 antibody-positive group were significantly higher than those in the anti-ARS antibody-positive group (P = 0.012 and 0.044, respectively). In contrast, the median scores of tortuous capillaries in the anti-ARS antibody-positive group were significantly higher than those in the anti-MDA5 antibody-positive group (P = 0.002). The median scores of microhemorrhage was significantly higher in the ILD-related death group than the survival group (P = 0.02). The scores of microhemorrhage, capillary disorganization, and neoangiogenesis correlated with known poor prognosis factors of DM-ILD. Additionally, the scores of microhemorrhage and capillary loss correlated significantly with the total fibrosis scores of chest high-resolution computed tomography. These findings suggest that NVC is a useful tool for assessing the disease activity and prognosis of DM-ILD.

scholarly journals Mesenchymal Stromal Cells for the Treatment of Interstitial Lung Disease in Children: A Look from Pediatric and Pediatric Surgeon Viewpoints

Cells ◽  
2021 ◽  
Vol 10 (12) ◽  
pp. 3270
Author(s):  
Gloria Pelizzo ◽  
Serena Silvestro ◽  
Maria Antonietta Avanzini ◽  
Gianvincenzo Zuccotti ◽  
Emanuela Mazzon ◽  
...  
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Mesenchymal Stromal Cells ◽  
Stromal Cells ◽  
Age Of Onset ◽  
Point Of View ◽  
High Morbidity ◽  
Severe Toxicity ◽  
Adult Age ◽  
Pulmonary Parenchyma

Mesenchymal stromal cells (MSCs) have been proposed as a potential therapy to treat congenital and acquired lung diseases. Due to their tissue-regenerative, anti-fibrotic, and immunomodulatory properties, MSCs combined with other therapy or alone could be considered as a new approach for repair and regeneration of the lung during disease progression and/or after post- surgical injury. Children interstitial lung disease (chILD) represent highly heterogeneous rare respiratory diseases, with a wild range of age of onset and disease expression. The chILD is characterized by inflammatory and fibrotic changes of the pulmonary parenchyma, leading to gas exchange impairment and chronic respiratory failure associated with high morbidity and mortality. The therapeutic strategy is mainly based on the use of corticosteroids, hydroxychloroquine, azithromycin, and supportive care; however, the efficacy is variable, and their long-term use is associated with severe toxicity. The role of MSCs as treatment has been proposed in clinical and pre-clinical studies. In this narrative review, we report on the currently available on MSCs treatment as therapeutical strategy in chILD. The progress into the therapy of respiratory disease in children is mandatory to ameliorate the prognosis and to prevent the progression in adult age. Cell therapy may be a future therapy from both a pediatric and pediatric surgeon’s point of view.

scholarly journals Coexistence of Axial Spondyloarthritis and Idiopathic Inflammatory Myopathy

2020 ◽  
Vol 2020 ◽  
pp. 1-4
Author(s):  
Yongpeng Ge ◽  
Linrong He
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Skin Rash ◽  
Axial Spondyloarthritis ◽  
Age Of Onset ◽  
Inflammatory Myopathy ◽  
Idiopathic Inflammatory Myopathy ◽  
Hla B27 ◽  
Laboratory Features ◽  
Friendship Hospital

Both axial spondyloarthritis (axSpA) and idiopathic inflammatory myopathy (IIM) are infrequent, and their coexistence is even rarer; there are a few reported cases in the literature. The aim of this study was to assess their association and clinical and laboratory features in our patients. The clinical data of patients with axSpA and IIM diagnosed in China-Japan Friendship Hospital from July 2015 to February 2019 were retrospectively analyzed. This study included 7 patients with axSpA who met the IIM criteria, including 3 males and 4 females. The age of onset was 16 to 39 years. Four patients were HLA-B27 positive, and three were negative. All patients were first diagnosed as axSpA, and then IIM was detected after 0.5–20 years (mean ± SD, 9.9 ± 5.0 years). After being diagnosed to have axSpA and IIM, those patients were given prednisone and immunosuppressant drugs, and their symptoms gradually improved. Our study provides further evidence of the coexistence of IIM with axSpA. In patients with axSpA who have skin rash, interstitial lung disease (ILD), myalgia, or muscle weakness, we should suspect that they may have IIM.

scholarly journals Clinical characteristics and pathogens of pediatric pyogenic liver abscess in Beijing Children’s Hospital, China

2020 ◽  
Author(s):  
Yue Xie ◽  
Ling-yun Guo ◽  
Lin-lin Liu ◽  
Bing Liu ◽  
Wen-ya Feng ◽  
...  
Keyword(s):  
Liver Abscess ◽  
Age Of Onset ◽  
Pyogenic Liver Abscess ◽  
Multiple Organ ◽  
Survival Group ◽  
Children's Hospital ◽  
Contrast Enhanced Ct ◽  
Diagnostic Time ◽  
Children’S Hospital ◽  
Death Group

Abstract Background: Data on pyogenic liver abscess (PLA) of children in China have been limited. Method: We retrospectively reviewed PLA cases from January 2017 to June 2019 at Beijing Children’s Hospital. Clinical features and pathogens were analyzed. Results: We diagnosed 15 PLA patients in our center. Median age of onset was 3.2 years and the median diagnostic time was 9 days. Four patients (26.7%) had underlying diseases and seven patients (46.7%) had multiple organ involvement. The sensitivity achieved 100% when ultrasound and contrast-enhanced CT scan were combined use to diagnose PLA. Two patients were defined pathogen through metagenomic next-generation sequencing (mNGS). Finally, eleven patients (73.3%) were cured during follow-up time and four patients (26.7%) dead. Compared to the survival group and the death group, the patients in death group were more likely to have multiple organ involved (100 vs. 27%, P < 0.05) and bilateral lobe occupied ( P < 0.05) . Conclusions: PLA often occurs in patients with underlying disease. Multiple organs involved may lead to poor prognosis. mNGS can be a new method to detect pathogen.

scholarly journals Presentations and outcomes of interstitial lung disease and the anti-Ro52 autoantibody

2019 ◽  
Vol 20 (1) ◽  
Author(s):  
A. Sclafani ◽  
K. M. D’Silva ◽  
B. P. Little ◽  
E. M. Miloslavsky ◽  
J. J. Locascio ◽  
...  
Keyword(s):  
Cohort Study ◽  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Retrospective Cohort ◽  
Specific Antibodies ◽  
Icu Admission ◽  
Clinical Presentations ◽  
All Cause Mortality ◽  
Specific Autoantibody ◽  
Poor Outcomes

Abstract Background Distinct clinical presentations of interstitial lung disease (ILD) with the myositis-specific antibodies, including anti-synthetase antibodies, are well-recognized. However, the association between ILD and the myositis-associated antibodies, including anti-Ro52, is less established. Our objectives were to compare presenting phenotypes of patients with anti-Ro52 alone versus in combination with myositis-specific autoantibodies and to identify predictors of disease progression or death. Methods We performed a retrospective cohort study of 73 adults with ILD and a positive anti-Ro52 antibody. We report clinical features, treatment, and outcomes. Results The majority of patients with ILD and anti-Ro52 had no established connective tissue disease (78%), and one-third had no rheumatologic symptoms. Thirteen patients (17.8%) required ICU admission for respiratory failure, with 84.6% all-cause mortality. Of the 73 subjects, 85.7% had a negative SS-A, and 49.3% met criteria for idiopathic pneumonia with autoimmune features (IPAF). The 50 patients with anti-Ro52 alone were indistinguishable from patients with anti-Ro52 plus a myositis-specific autoantibody. ICU admission was associated with poor outcomes (HR 12.97, 95% CI 5.07–34.0, p < 0.0001), whereas rheumatologic symptoms or ANA > = 1:320 were associated with better outcomes (HR 0.4, 95% CI 0.16–0.97, p = 0.04, and HR 0.29, 95% CI 0.09–0.81, p = 0.03, respectively). Conclusions Presentations of ILD with the anti-Ro52 antibody are heterogeneous, and outcomes are similar when compared to anti-Ro52 plus myositis-specific antibodies. Testing for anti-Ro52 may help to phenotype unclassifiable ILD patients, particularly as part of the serologic criteria for IPAF. Further research is needed to investigate treatment of ILD in the setting of anti-Ro52 positivity.

scholarly journals The Role of Radiology in Progressive Fibrosing Interstitial Lung Disease

2022 ◽  
Vol 8 ◽  
Author(s):  
Ahmad Abu Qubo ◽  
K. M. Capaccione ◽  
Elana J. Bernstein ◽  
Maria Padilla ◽  
Mary Salvatore
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Clinical Diagnosis ◽  
Early Death ◽  
Radiographic Diagnosis ◽  
Increased Risk

In this article, we describe the role of radiology for diagnosis and follow-up of progressive fibrosing interstitial lung disease (PF-ILD). Patients with PF-ILD are at increased risk for early death without treatment. Clinical diagnosis of PF-ILD has been described in the literature. This manuscript reviews the radiographic diagnosis of PF-ILD and the unique CT characteristics associated with specific types of fibrosis. Ultimately, we believe that radiology has the potential to recognize progression early and thus make an important contribution to the multidisciplinary discussion for this important diagnosis.

scholarly journals KL-6 as an Immunological Biomarker Predicts the Severity, Progression, Acute Exacerbation, and Poor Outcomes of Interstitial Lung Disease: A Systematic Review and Meta-Analysis

2021 ◽  
Vol 12 ◽  
Author(s):  
Tao Zhang ◽  
Ping Shen ◽  
Chunyan Duan ◽  
Lingyun Gao
Keyword(s):  
Interstitial Lung Disease ◽  
Lung Disease ◽  
Acute Exacerbation ◽  
Adverse Outcome ◽  
Weighted Mean Difference ◽  
Meta Analysis ◽  
Effect Sizes ◽  
Weighted Mean ◽  
Poor Outcomes ◽  
The Relationship

ObjectInterstitial lung disease (ILD) is a specific form of chronic fibrosing interstitial pneumonia with various etiology. The severity and progression of ILD usually predict the poor outcomes of ILD. Otherwise, Krebs von den Lungen-6 (KL-6) is a potential immunological biomarker reflecting the severity and progression of ILD. This meta-analysis is to clarify the predictive value of elevated KL-6 levels in ILD.MethodEBSCO, PubMed, and Cochrane were systematically searched for articles exploring the prognosis of ILD published between January 1980 and April 2021. The Weighted Mean Difference (WMD) and 95% Confidence Interval (CI) were computed as the effect sizes for comparisons between groups. For the relationship between adverse outcome and elevated KL-6 concentration, Hazard Ratio (HR), and its 95%CI were used to estimate the risk factor of ILD.ResultOur result showed that ILD patients in severe and progressive groups had higher KL-6 levels, and the KL-6 level of patients in the severe ILD was 703.41 (U/ml) than in mild ILD. The KL-6 level in progressive ILD group was 325.98 (U/ml) higher than that in the non-progressive ILD group. Secondly, the KL-6 level of patients in acute exacerbation (AE) of ILD was 545.44 (U/ml) higher than stable ILD. Lastly, the higher KL-6 level in ILD patients predicted poor outcomes. The KL-6 level in death of ILD was 383.53 (U/ml) higher than in survivors of ILD. The pooled HR (95%CI) about elevated KL-6 level predicting the mortality of ILD was 2.05 (1.50–2.78), and the HR (95%CI) for progression of ILD was 1.98 (1.07–3.67).ConclusionThe elevated KL-6 level indicated more severe, more progressive, and predicted the higher mortality and poor outcomes of ILD.

scholarly journals Correlation Analysis between Mechanical Power and Lung Ultrasound Score and Their Evaluation of Severity and Prognosis in ARDS Patients

2021 ◽  
Vol 2021 ◽  
pp. 1-6
Author(s):  
Yongpeng Xie ◽  
Suxia Liu ◽  
Zhifang Mou ◽  
Yanli Wang ◽  
Xiaomin Li
Keyword(s):  
Mechanical Ventilation ◽  
Correlation Analysis ◽  
Lung Ultrasound ◽  
Lung Ventilation ◽  
Mechanical Power ◽  
Apache Ii Score ◽  
Survival Group ◽  
Time Points ◽  
Death Group ◽  
Time Point

Background and Objective. During mechanical ventilation, the energy delivered by a ventilator to the respiratory system in one minute is defined as mechanical power (MP). It describes the combined effects of several mechanical factors, including tidal volume, driving pressure, transpulmonary pressure, flow rate, positive end-expiratory pressure, and respiratory rate on lung ventilation. As such, MP may be useful for evaluating the severity and prognosis of acute respiratory distress syndrome (ARDS), as improvements in pulmonary ventilation directly affect a patient’s prognosis. Recent advances in lung ultrasound technology make it possible to evaluate lung ventilation at the bedside. In this study, MP was monitored in ARDS patients, and its correlation with lung ultrasound scores (LUS) was evaluated. Further analysis also explored the value of MP in evaluating the severity and prognosis of ARDS. Methods. A total of 121 patients with moderate to severe ARDS admitted to the intensive care unit (ICU) from June 2017 to April 2020 and treated with invasive mechanical ventilation were sequentially included in this study. Their general information was collected, and MP was recorded at 0 h, 24 h, 48 h, and 72 h after admission to the ICU. Professionally trained researchers performed the LUS assessments. Patients were divided into the death and survival groups according to their 28-day prognosis. The trend of MP and LUS at the four time points was analyzed. A receiver operating characteristic curve (ROC) was used to analyze the predictive value of MP and LUS scores at 0 h and 72 h for the prognosis (28-day mortality rate) of patients with moderate to severe ARDS. Results. 121 patients were included in the analysis, of which 73 were male and 48 were female. When patients entered the ICU, their oxygenation index ( t : 30885, P < 0.01 ), APACHE II score ( t : 2.105, P < 0.05 ), and SOFA score ( t : 4.134, P < 0.001 ) were higher in the death group than the survival group. The death group had significantly higher MP and LUS at each time point (0 h, 24 h, 48 h, and 72 h) compared to the survival group (all P < 0.05 ). There was a significant upward trend over time in the MP and LUS of the death group, contrasting to a significant downward trend in the survival group (all P < 0.05 ). The Pearson correlation analysis showed that MP and LUS were significantly positively correlated at each time point ( r values: 0 h: 0.3027; 24 h: 0.3705; 48 h: 0.3902; 72 h: 0.5916; all P < 0.01 ). The ROC curves showed that MP and LUS at 72 h were of significant value in predicting the prognosis of ARDS patients, with areas under the curve of 0.866 ± 0.032 and 0.839 ± 0.037 , respectively. Conclusion. There was a significant correlation between the MP and LUS of ARDS patients at four time points from 0 to 72 h, which has a clinical value in evaluating severity and prognosis.

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