ObjectiveTo translate the King’s Brief Interstitial Lung Disease (K-BILD) to Chinese, so as to provide an well reliability and validity assessment instrument for health status of patients with interstitial lung disease.MethodsBrislin’s transition model, six expert’s panel and pre-survey were used for initial Chinese version of K-BILD. Items analysis, exploratory factor analysis (EFA), confirmatory factor analysis (CFA), internal consistency reliability and test-retest reliability were used for validity and reliability test with 122 respondents.ResultsTen-item Chinese version of K-BILD were proved to have great psychometric qualities, two factors were extracted by EFA, which could explain 63.35% of the total variance. Furthermore, the CFA demonstrates the fit indices of two-factors mode: χ2/df=0.797, RMSEA=0.000, NFI=0.848, IFI=1.048, CFI=1.000, TLI=1.071. Cronbach’s α and Guttman Split-half were 0.893 and 0.861, respectively. Besides, the test-retest reliability of the scale was 0.805.ConclusionThe Chinese version of K-BILD scale has good validity and reliability, which is applicable for health status assessment in patient with interstitial lung disease.
ObjectiveTo analyze the benefits of lung transplantation in the treatment of interstitial lung disease (ILD) and investigate its prognostic factors.MethodsThe clinical data of patients diagnosed with ILD and meet the lung transplantation criteria were retrospectively analyzed from January 2012 to December 2017 in the First Affiliated Hospital of Guangzhou Medical University. A total of 111 patients, 88 males and 23 females, aged (58.3±11.4) years old, were divided into lung transplantation group and non-lung transplantation group. Clinical data and prognosis of the two groups were compared and the factors affecting the prognosis of lung transplantation were analyzed with relevant literatures. Results There were 56 patients in lung transplantation group and 55 patients in non-lung transplantation group. The mainly underlying disease of both groups were idiopathic pulmonary fibrosis (IPF). There was no significant difference in age, body mass index, arterial partial pressure of oxygen, percentage of forced vital capacity in the estimated value, percentage of diffusing capacity of the lung for carbon monoxide in the estimated value, six-minute walk distance between the two groups (P>0.05). The pulmonary arterial hypertension and arterial partial pressure of carbondioxide were higher in lung transplantation group than non-transplantation group (P<0.05). The 1-year survival rate in the lung transplantation group was significantly higher than that in the non-lung transplantation group: 77.4% vs. 32.7% (P<0.01). COX regression analysis showed that preoperative ventilator dependence, serum creatinine, bilirubin, pulmonary artery pressure, and procedures (single lung vs. double lung) had no significant effect on the prognosis of lung transplantation; age and preoperative diabetes mellitus were risk factors for the prognosis of lung transplantation.ConclusionsLung transplantation can significantly improve the prognosis of patients with ILD who are refractory to medicine therapy. IPF patients should be advised to consider lung transplantation as soon as possible. Age and preoperative diabetes mellitus are risk factors for the prognosis of lung transplantation.
ObjectiveTo explore the therapeutic effects of spleen aminopeptide on connective tissue disease-related interstitial lung disease (CTD-ILD) and its mechanism for anti-fibrosis. MethodsNinety patients with CTD-ILD admitted between February 2014 and May 2015 were recruited in the study. The CTD-ILD patients were randomly divided into group A (conventional therapy alone) and group B (conventional therapy plus spleen aminopeptide). Peripheral blood collected from CTD-ILD patients were subjected to performance of flow cytometric analysis and cytokine/chemokines profiling by liquid Chip and ELISA assay. Pulmonary function test and high resolution CT (HRCT) scan were performed before and after the treatments for 12 weeks. Human cytomegalovirus (HCMV) DNA in the patients' blood was tested by Q-PCR. ResultsSignificantly improved lung function and HRCT score were observed in group B, but not in group A. The levels of Treg and IFN-γ were significantly increased in group B, compared with those in group A where markedly increased IL-6, IL-10 and IL-17 were detected (P < 0.05). There was higher virus negative reversal rate in group B than that in group A (P < 0.05). ConclusionSpleen aminopeptid can effectively regulate deregulated immune microenvironment in CTD-ILD patients and inhibit HCMV replication, thereby block pulmonary fibrotic development.
Objective To summarize the clinical characteristics of interstitial pneumonia with autoimmune features (IPAF). Methods The interstitial lung disease (ILD) patients diagnosed in our department between January 2010 and August 2013 were retrospectively analyzed to screen out the patients with IPAF.The clinical manifestations, laboratory examination, imaging, pulmonary function and treatment were summarized. Results In 254 ILD patients, 25 patients met the diagnosis criteria of IPAF, and 26 patients were diagnosed with definite connective tissue diseases associated ILD (DCTD-ILD). There were differences in arthralgia, sicca symptoms, mechanic’s hand, positive antinuclear antibodies, anti-CCP antibodies and residual lung volume between the IPAF patients and the DCTD-ILD patients (all P < 0. 05). Five IPAF patients were revealed hug or “pancake” the diaphragm in their chest high resolution CT radiographs. The microscopic performance showed that diffuse thickened with collagen fiber, alveolar wall thickening with marked interstitial lymphocyte inflammatory cells infiltration, and granulation tissue that filled bronchiolar lumina. The patients were pathologically diagnosed with nonspecific interstitial pneumonia (NSIP) overlap organized pneumonia (OP). During following-up, the progression-free survival time of the IPAF patients was significant longer and that of the DCTD-ILD patients [(14.32±5.74)months vs. (10. 31± 3. 70) months, P < 0. 05]. Conclusions If an ILD patient has mechanic’s hand, positive antinuclear antibodies or NISP overlap OP in image, the diagnosis of IPAF should be considered. IPAF have slower disease progression and better prognosis than DCTD-ILD.
Systemic lupus erythematosus is an autoimmune disease involving multiple organs of the body. Lupus nephritis is one of the most serious organ manifestations of systemic lupus erythematosus. Vimentin, a member of the intermediate filament protein family, is involved in the pathogenesis of many autoimmune diseases, including lupus nephritis. More and more studies have shown that vimentin plays an important role in the pathogenesis of lupus nephritis, and has an important influence on the disease development, treatment and prognosis of lupus nephritis. This review focuses on the structure, function and post-translational modification of vimentin, the relationship between vimentin and the pathogenesis of lupus nephritis, and the significance of vimentin expression levels in renal tissues, serum and urine, in order to provide theoretical basis for future mechanism research and clinical application.
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.
ObjectiveTo analyze the CT features of immune checkpoint inhibitor-related pneumonia (CIP) and improve the diagnostic accuracy of CIP. MethodsAmong patients with malignant tumor treated with immune checkpoint inhibitors, those who developed pneumonia and rule out other causes of disease were identified. Chest CT Imaging were reviewed to assess special signs, distribution characteristics, severity of pneumonia and radiographic patterns of CIP. ResultsA total of 28 patients were enrolled, including 26 males and 2 females. CT features include ground-glass opacity, centrilobular nodularity, reticular opacity, consolidation, traction bronchiectasis, honeycomb, etc. The lesions predominant involved peripheral lung zone (17/28), lower lung zone (18/28) and posterior lung zone (18/28), with a diffuse distribution (23/28). In most cases the disease involved both lungs (23/28), and a few involved unilateral or single lobe. The most common affected lobes were the lower lobe of the right lung (25/28) and the lower lobe of the left lung (20/28), followed by the upper lobe of the right lung (18/28). Mean pneumonia severity score was 5.5, standard deviation was 3.8, and range was 1 - 15. The most common radiographic patterns of CIP were nonspecific interstitial pneumonia (11/28) and hypersensitivity pneumonia (10/28). The second was organizing pneumonia (6/28). ConclusionsThe CT manifestations of CIP have certain specificity. Combined with the history of drug treatment and clinical symptoms of patients, the early and correct diagnosis can be obtained.
ObjectiveTo investigate the prognostic relevance of serum triglyceride (TG) levels in patients with anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive dermatomyositis-associated interstitial lung disease (ILD). Methods A retrospective data collection was conducted on patients diagnosed with anti-MDA5 antibody-positive dermatomyositis-associated ILD at West China Hospital of Sichuan University between February 2017 and July 2021. The clinical data, laboratory tests, and imaging examinations were collected, and the patients were followed up. According to the survival and death status of patients, they were divided into survival group and death group. According to TG levels, the patients were divided into a TG high level group and a TG low level group. We employed Cox proportional hazard models to investigate the variables linked to the mortality of individuals afflicted with anti-MDA5 antibody-positive dermatomyositis-associated ILD. Results A total of 204 patients with anti-MDA5 antibody-positive dermatomyositis-associated ILD were included. Among them, whose age ranged from 30 to 81 years old, with an average of (49.5±11.8) years old, there were 69 males and 135 females, 53 deaths and 151 survivors, 57 cases of rapidly progressive pulmonary interstitial fibrosis (RPILD) and 47 cases of non-RPILD. The results of multivariate Cox regression analysis showed that TG≥1.65 mmol/L, combined with RPILD, combined with dyspnea, age, lactate dehydrogenase≥321 U/L, and albumin<30 g/L were independent factors affecting the long-term prognosis of patients (P<0.05). The Kaplan-Meier method analysis results showed that the survival rate of the TG high level group was lower than that of the TG low level group (P=0.032). Conclusions Elevated TG levels can serve as a clinical indicator of adverse prognosis in patients with dermatomyositis-associated ILD who exhibit positive anti-MDA5 antibody status. Additionally, age, comorbidity with RPILD, combined with dyspnea, lactate dehydrogenase≥321 U/L, and albumin<30 g/L are independent factors contributing to the increased mortality risk among individuals with dermatomyositis-associated ILD who test positive for anti-MDA5 antibody.