Interstitial lung disease is the most common pulmonary complication in patients with inflammatory myopathy, with a high case fatality rate, unknown pathogenesis, and complex clinical manifestations, and the treatment is difficult. Early and timely treatment can improve the patient’s clinical symptoms and inhibit the development of the disease. The present treatment protocols can be mainly summarized as the commonly used drugs (corticosteroids, azathioprine, cyclophosphamide, mycophenolate mofetil, and intravenous immunoglobulin) and new drugs (cyclosporin A, tacrolimus, biological agents, and anti-fibrosis drug), etc. In this paper, the treatment progress of inflammatory myopathy-related interstitial lung disease and different myositis antibody-related interstitial lung disease in recent years at home and abroad is reviewed, so as to provide a basis for clinical treatment.
Objective To explore the effects of bone marrow mesenchymal stem cells ( BMMSCs) on pulmonary fibroblasts of patients with nonspecific interstitial pneumonitis ( NSIP) , and investigate the therapeutic mechanism of BMMSCs for interstitial pulmonary fibrosis. Methods Human BMMSCs, human pulmonry fibroblasts ( HPFs) from NSIP patients, and normal HPFs were primary cultured in vitro. Then HPFs fromNSIP patients were co-cultured with BMMSCs or normal HPFs using Transwell co-culture system. After 24 hours, levels of transforming growth factor β1 (TGF-β1) and interferon inducible protein 10 ( IP-10) in culture supernatants were detected by ELISA method. Meanwhile, interleukin-6 ( IL-6) , IL-8, and monocyte chemotactic protein-1 ( MCP-1) in co-culture supernatants were detected by liquid chip. After co-cultured for 48 hours, total protein of HPFs was extracted and the expression level of alpha smooth muscle actin ( α-SMA) secreted by HPFs were detected by Western blot.Results HPFs from NSIP patients secreted higher level of IL-6, IL-8, and MCP-1 than normal HPFs, and secreted high level of α-SMA. In the Transwell co-culture system, human BMMSCs significantly reduced the levels of IL-6, IL-8, and MCP-1 secreted from HPFs of NSIP patients, and reduced the high expression of α-SMA in HPFs of NSIP patients. Conclusion Human BMMSCs can significantly reduce the secretion of IL-6, IL-8, MCP-1, and the expression of α-SMA in HPFs from NSIP patients.
ObjectiveTo analyze the clinical radiographic features and treatment of interstitial lung disease (ILD) inpatients infected with influenza virus. MethodsThe clinical data of ILD patients with influenza in Nanjing Drum Tower Hospital from October 2023 to January 2024 were collected. According to each patient results of influenza nucleic acid detection, they were divided into an influenza infection group and a non-infection group. ResultsA total of 73 patients received influenza nucleic acid detection were enrolled, 23 cases including 5 males and 18 females were positive. Twenty-one cases were infected with influenza A virus, 2 cases were infected with influenza B virus. The median age of influenza positive patients was 64.7±7.8 years. Cough (23 cases, 100.0%), sputum (23 cases, 100.0%), wheezing (20 cases, 87.0%) and fever (17 cases, 73.9%) were the most common symptoms of the patients infected with influenza. Compared with the non-infection patients, fever was more common in the influenza infection group (P<0.05). Laboratory examination indicated that lymphocytopenia were detected in the influenza infection patients. There was no statistical difference in the level of white blood cell count, neutrophil count, lactate dehydrogenase, creatine kinase, erythrocyte sedimentation rate, C-reactive protein, calcitonin, interleukin-6 and oxygenation index. Ground glass opacity in the influenza virus infection group was more common than that in the non-infection group (P<0.05). Ten ILD patients infected with influenza virus (43.5%) were co-infected with Aspergillus. The chest CT type of ILD patients with Aspergillus infection was usual interstitial pneumonia (UIP). Honeycombing was more common than those without Aspergillus infection group (P<0.05). Twenty-two patients (95.7%) received antiviral treatment, of which 20 patients (87.0%) were treated with oseltamivir, 5 patients (21.7%) were treated with mabaloxavir, and 4 patients (17.4%) were treated combined with paramivir. Seventeen patients (73.9%) were previously treated with glucocorticoids, and 16 patients did not adjust the glucocorticoids dosage; 9 patients (39.1%) were previously treated with immunosuppressants, and 2 patients stopped immunosuppressants. Four patients (17.4%) infected with influenza virus developed acute exacerbation of ILD. There was no statistically significant difference in acute exacerbation between the two groups (P>0.05). ConclusionsCompared with ILD patients not infected with influenza, fever, lymphocytopenia and ground-glass opacity are the common clinical and chest CT features of ILD patients infected with influenza. Patients with UIP type combined with honeycomb were prone to be co-infected with Aspergillus infection.
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 incidence of heart disease, its clinical manifestations and risk factors in patients with polymyositis (PM) and dermatomyositis (DM). MethodWe collected the clinical data of 138 PM (n=78) and DM (n=60) patients treated between January 2008 and March 2014, among whom there were 64 males and 74 females with an average age of (48.5±19.6) years old, and an average disease course of (84.5±6.9) months. We analyzed their incidence of heart disease, its clinical manifestations and the risk factors. Subsequently, we adopted single-factor analysis to analyze such factors as age, gender, disease course, kinds of disease, creatine kinase (CK), CK-MB, CK-MB/CK, troponin T, antinuclear antibodies, anti-SSA antibody, erythrocyte sedimentation rate, C-reactive protein, disease activity score, muscle force, pulmonary interstitial lesions and pulmonary artery hypertension. ResultsThere were 59 (42.7%) patients with heart disease, 48 (34.8%) with abnormal electrocardiogram, and 52 (37.7%) with abnormal color Doppler ultrasound results. Logistics multiple factors regression analysis found that the course of the disease (OR=1.669, P=0.010), myositis disease activity score (OR=7.456, P<0.001), pulmonary interstitial lesions (OR=4.568, P=0.014) were risk factor for heart disease in PM/DM patients. ConclusionsLong disease course duration, high myositis activity score and pulmonary interstitial lesions are strong predictors for heart damage in PM/DM patients.
Objective To evaluate the diagnostic value and safety of surgical lung biopsies ( SLB)in patients with interstitial lung disease ( ILD) . Methods A retrospective analysis was performed on patients undergoing SLB, who were obtained from Chinese literatures through searching PubMed, CBM,Wanfang database, VIP information and CHKD from 2000 to 2010. The data from Affiliated Drum Tower Hospital, Nanjing University Medical College from2000 to 2010 was also reviewed. Results A total of 398cases underwent SLB, including open lung biopsy ( OLB) in 221 cases and video-assisted thoracoscopic lung biopsy ( VATLB) in 177 cases. SLB yielded a specific diagnosis for 386 cases. The total postoperative complication rate was 12. 1% and mortality rate was 2. 0% . The diagnostic yield, post-operative complication rate, and mortality rate between VATLB and OLB had no significant difference. Conclusion SLB is a useful and relatively safe procedure for diagnosis of ILD.
Objective To summarize the clinical characteristics of pneumocystis pneumonia (PCP) secondary to interstitial lung disease (ILD) to improve the prophylaxis and management level of clinicians. Methods The clinical data of 50 patients with PCP secondary to ILD in the Department of Respiratory and Critical Care Medicine of Nanjing Drum Tower Hospital from January 2015 to December 2022 were collected. SPSS 26.0 software was used for statistical analysis. Results A total of 50 patients with PCP secondary to ILD were screened. Among the 50 patients, there were 23 males and 27 females, with a median age of 64 years old. Forty-eight cases (96%) had a history of glucocorticoid therapy with the median duration of 3 months; 31 (77.5%, 31/40) cases developed PCP in the first 6 months after glucocorticoid therapy; 34 cases had a history of glucocorticoid and immunosuppressants at the same time. None of the 50 ILD patients used drugs for PCP prophylaxis before developing PCP. The major clinical manifestations of PCP secondary to ILD were worse cough and shortness of breath or fever. Laboratory results showed 38 cases (76.0%) had peripheral blood total lymphocyte count <200/µL, 27 cases (54.0%) had CD4+ T cell count <200/µL, 34 cases (68.0%) had CD4+ T cell count <300/µL, 37 cases (74.0%) had CD3+ T cell count <750/µL, 34 cases (68.0%) had β-D-glucan test >200 pg/mL, 35 cases (70.0%) had lactic dehydrogenase > 350 U/L and 41 cases (82.0%) had type Ⅰ respiratory failure. High resolution computed tomography showed added ground-glass opacity and consolidation on the basis of the original ILD. Thirty-six cases were detected the Pneumocystis jirovecii by metagenomic next-generation sequencing with broncho-alveolar lavage fluid as the main source, and 2 cases by smear microscopy. All patients were treated with trimethoprim-sulfamethoxazole. After treatment, 29 cases were discharged with a better health condition, 10 cased died, and 11 cases left hospital voluntarily because of treatment failure or disease deterioration. Conclusions After the use of glucocorticoid and immunosuppressants, ILD patients are susceptible to life-threatening PCP. It is particularly important to make an early diagnosis. Attention should be paid to integrate the symptoms, levels of peripheral blood lymphocyte count, β-D-glucan test, lactic dehydrogenase and imaging findings to make an overall consideration. It is suggested to perform next-generation sequencing with broncho-alveolar lavage fluid at an early stage when patients can tolerate fiberoptic bronchoscopy to avoid misdiagnosis and missed diagnosis. ILD patients often develop PCP in the first 6 months after using glucocorticoid and immunosuppressants. During follow-up, peripheral blood CD4+ and CD3+ T cell count should regularly be monitored so as to timely prevent PCP.
Objective To investigate the levels and clinical significance of cold-inducible RNA-binding protein (CIRBP) and mucin 5AC (MUC5AC) in serum and bronchoalveolar lavage fluid (BALF) of patients with interstitial lung disease (ILD). Methods A total of 63 ILD patients who were hospitalized in the Department of Pulmonary and Critical Care Medicine, The First Hospital of Lanzhou University from January 2022 to February 2024 were collected, including 22 patients with idiopathic pulmonary fibrosis (IPF), 20 patients with connective tissue disease-related interstitial lung disease (CTD-ILD), and 21 patients with other types of ILD. Thirty healthy individuals with matching demographic characteristics during the same time period were selected as the healthy group. Enzyme linked immunosorbent assay was used to detect the levels of CIRBP and MUC5AC in serum of healthy group, the levels of CIRBP and MUC5AC in serum and BLAF of ILD patients. The levels of CIRBP and MUC5AC in serum were compared between the healthy group and the ILD group, and the levels of CIRBP and MUC5AC in serum and BALF were compared among the patients with different types of ILD. The correlations were analyzed between the levels of CIRBP and MUC5AC and the lung function, blood gas analysis, and 6-minute walk distance (6MWD) in the patients with different types of ILD. The receiver operating characteristic curve was drawn according to the levels of CIRBP and MUC5AC in serum. The optimal cut-off value, sensitivity and specificity of serum CIRBP and MUC5AC for diagnosis of ILD were determined. Results The serum CIRBP level in the three groups of ILD patients was higher than that of the healthy group (P<0.01), but there was no significant difference in the serum CIRBP level among the three groups of ILD patients (P>0.05). There was no significant difference in serum MUC5AC levels between the three groups of ILD patients and the healthy group, as well as among the three groups of ILD patients (P>0.05). There was no significant difference in the levels of CIRBP and MUC5AC in BALF among the three groups of ILD patients (P>0.05). CIRBP in BALF of the IPF patients was negatively correlated with 6MWD, while CIRBP in serum of the CTD-ILD patients was negatively correlated with partial pressure of oxygen and arterial oxygen saturation. CIRBP in serum of other ILD patients was negatively correlated with total lung capacity as a percentage of predicted value (TLC%pred), forced vital capacity as a percentage of predicted value (FVC%pred), diffusion capacity of carbon monoxide of lung as a percentage of predicted value (DLCO%pred), and 6MWD, while CIRBP in BALF was negatively correlated with DLCO%pred. The MUC5AC in BALF of IPF patients was negatively correlated with DLCO%pred, while the MUC5AC in BALF of CTD-ILD patients was negatively correlated with TLC%pred, DLCO%pred, and 6MWD. The serum MUC5AC in other ILD patients was negatively correlated with FVC%pred, DLCO% pred, and 6MWD. The sensitivity was 77.6%, and the specificity was 96.7% for diagnosing ILD with serum CIRBP. Sensitivity was 55.1%, and specificity was 76.7% for diagnosing ILD with serum MUC5AC. The combination of the two indicators resulted in a sensitivity of 87.8% and a specificity of 86.7% for the diagnosis of ILD. Conclusion The high expression levels of CIRBP and MUC5AC have certain diagnostic efficacy and disease assessment effects on ILD, and may serve as potential biomarkers for ILD.
Idiopathic inflammatory myopathies are a group of connective tissue diseases characterized by nonsuppurative inflammation of the striated muscle. At present, the diagnostic criteria for polymyositis/dermatomyositis classification proposed by Bohan and Peter in 1976 is mainly used clinically. In clinical observations, it is found that myopathy involves not only skin and muscle but also affects many systems of the body. Interstitial lung disease occupies an important part, and it is an important cause of death of patients with inflammatory myopathy. Patients with idiopathic myositis should be examined as early as possible by high-resolution CT to improve the detection rate of myositis-associated interstitial lung disease and start treatment as soon as possible. At the same time, the patients with myositis have different clinical manifestations due to specific antibodies in the serum; some specific antibodies may indicate poor prognosis and poor treatment response. Timely screening of patients with positive myositis-specific antibodies in patients with the pulmonary interstitial disease can help the patient’s diagnosis and treatment process.
Melanoma differentiation associated gene 5 (MDA5) antibody is a kind of myositis-specific autoantibodies, which can be used as a biological marker for dermatomyositis (DM) patients. It has been shown that this antibody is closely related to the occurrence of interstilung disease (ILD) in DM patients, which may lead to rapidly progressive interstitial lung disease in DM patients with MDA5 antibody positive, leading to a higher mortality rate. Therefore, the early detection, diagnosis and treatment of this disease have attracted the attention of clinical workers. In this paper, the progress of diagnosis and treatment of MDA5 antibody-positive DM with ILD is reviewed.