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.
ObjectiveTo evaluate the prevalence of interstitial lung disease (ILD) in patients with rheumatoid arthritis (RA),and explore the association of ILD with RA. MethodsPatients who were diagnosed as RA were recruited in the study,and were categorized into a RA-ILD group and a non-ILD group according to high-resolution computed tomography (HRCT).The data of clinical characteristics,serum biomarkers measurement,and pulmonary function test were collected. Logistic regression analysis was performed to evaluate the factors associated with the incidence of ILD in RA. ResultsA total of 59 patients with RA were enrolled. The incidence of ILD in RA was 42.3%. The rheumatoid factor (RF),carbohydrate antigen 15-3 (CA15-3),carbohydrate antigen12-5 (CA125),inspiratory capacity (IC) and diffusion capacity for carbon monoxide of the lung (DLCO) were significantly different between two groups (all P<0.05). Multivariate logistic regression analysis revealed that the increased CA15-3 and decreased DLCO were independent risk factors for ILD in the patients with RA. ConclusionsThe occurrence of ILD with RA seems very high. CA15-3 and DLCO may be important risk factors for the incidence of ILD in patients with RA.
ObjectiveTo determine the diagnostic value of serum KL-6 level in patients with interstitial lung diseases (ILD). MethodsAll the ILD patients enrolled were hospitalized from April 2013 to April 2014. Patients with other pulmonary diseases and healthy subjects were chosen as control groups simultaneously. Serum KL-6 concentrations were measured by chemiluminescent enzyme immunoassay. The association with serum KL-6 level and pulmonary function was analyzed. ResultsThere were 149 ILD patients, 155 patients with other pulmonary diseases, and 64 healthy subjects. The average serum levels of KL-6 were (1 801.86±2 831.36) U/mL, (267.00±124.41) U/mL, (201.28±81.18) U/mL in the patients with ILD, the patients with other pulmonary diseases and the healthy controls, respectively. The sensitivity and the specificity of the serum KL-6 for the diagnosis of ILD was 83.89% and 92.24% respectively when the cut-off level was set at 500 U/mL. The Kappa value was 0.767 (P < 0.001). The best cut-off value of KL-6 was 469.5 U/mL. Serum KL-6 levels in the patients with ILD were significantly higher compared with the patients with chronic obstructive pulmonary disease, pneumonia, tuberculosis, bronchiectasis and the healthy controls, respectively (all P < 0.001). The KL-6 levels in the pulmonary alveolar proteinosis patients were significantly higher compared with the patients with cryptogenic organizing pneumonia (COP), the patients with idiopathic pulmonary fibrosis (IPF) and the patients with connective tissue disease (CTD-ILD) (all P < 0.001). While the KL-6 concentration in IPF and CTD-ILD were significantly higher than that in COP (P=0.003 and P=0.008, respectively). Significant negative correlations were found between the levels of serum KL-6 and vital capacity as a percentage of the predicted value, forced vital capacity as a percentage of the predicted value, forced expiratory volume in one second as a percentage of the predicted value and carbon monoxide diffusing capacity as a percentage of the predicted value (all P < 0.001). Follow-up study showed the levels of serum KL-6 were consistent with clinical efficacy. ConclusionSerum KL-6 level is a reliable serum marker for ILD, and is related with the severity of disease and clinical efficacy.
Objective To investigate the lung involvement in Chinese patients with systemic sclerosis ( SSc) and its functional impact. Methods 68 patients with SSc were enrolled in Scleroderma study of PUMCH ( Peking Union Medical College Hospital) . All the patients underwent high resolution computed tomography ( HRCT) , pulmonary function testing, 6-minute walk testing, and echocardiography. And 36 patients filled in the Saint George’s Respiratory Questionnaire ( SGRQ) for assessment of healthrelated quality of life. Results HRCT revealed interstitial lung disease ( ILD) in 52 ( 76. 5% ) patients, 20 out of them without respiratory symptoms. Reticular opacification, ground-glass opacity ( GGO) , traction bronchiectasis, and honeycomb were presented respectively in 80.8% , 73.1% , 59.6% , and 30.8% of patients with SSc-ILD. Fibrosis was the predominant HRCT finding, and pure GGO ( in the absence of reticulation or architectural distortion) was only present in 8 ( 15. 7% ) patients. 57 (83.8% ) patients presented with diffusion defect, with most of them having moderate to severe impairent. Reduced FVC or TLC presented in 20 ( 29.4% ) and 28 ( 41.2% ) of patients respectively. The significant correlation was observed between the DLCO and the extent of ILD on HRCT ( rs = -0.476, P =0.000) . DLCO showed significant correlations with all the four components of the SGRQ ( Plt;0.05) . Significant correlations were also observed among the SGRQ scores and SpO2 ( maximum desaturation) or Borg index. Stepwise multiple regression analysis confirmed that the DLCO, SpO2 , and Borg index contributed to the SGRQ. Conclusions Lung involvement in Chinese SSc patients is common and insidious. The HRCT features of SSc-ILD are predominant fibrosis plus GGO, indicating little reversibility. Thus HRCT should be performed routinely in newly diagnosed SSc patients for early screening of lung involvement. The lung function defect of SSc is characterized by reduced diffusing capacity, and DLCO show better correlations with HRCT abnormities or SGRQ than FVC or TLC. Thus DLCO is of great value for early detection or severity assessment of SSc-ILD. SGRQ can be used to examine the health related quality of life of SSc patients and may reflect severity of lung involvement.
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 investigate the correlation between brain natriuretic peptide (BNP) and cardiopulmonary function, as well as explore prognostic value of BNP in patients with interstitial lung disease(ILD). MethodsThe clinical data of ILD patients admitted between February 2010 and April 2013 were retrospectively analyzed.The relationship between serum BNP level and cardiopulmonary function was analyzed by Pearson's or Spearman's rank correlation test.Meanwhile, the patients were divided into a survivor group and a death group, as well as a pulmonary hypertention (PH) group and a normal group respectively.Clinical data were compared between two groups. ResultsThere were 38 patients included in the study.BNP concentration was correlated with echocardiographic indices of right heart function, including systolic pressure of pulmonary artery, right atrial inlet diameter, right ventricular inlet diameter, and right ventricular end-diastollc diameter (P < 0.05), but not correlated with echocardiographic parameters of left heart function (P > 0.05).BNP was also correlated with DLCO%pred.BNP and right heart function parameters except right atrial inlet diameter were significantly higher, and DLCO%pred, were significantly lower in the death group and the PH group than those in the survivor group and the normal group (P < 0.05). ConclusionsSerum BNP concentration can reflect right heart function appropriately, suggesting pulmonary vascular impairment in ILD patients contributes to increased pulmonary vascular resistance.Elevated BNP levels are associated with increased mortality and poor prognosis in patients with ILD.
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 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.
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 This study aims to analyze the clinical characteristics of idiopathic inflammatory myopathy (IIM) associated interstitial lung disease (ILD) with positive anti melanoma differentiation-associated gene 5 (MDA5) antibody. Methods The clinical data of 91 patients with IIM in Shanxi Bethune Hospital from Jan 2019 to Mar 2022 were collected. Then various comparisons and analyzed retrospectively in the clinical symptoms, laboratory indicators and imaging characteristics between the negative MDA5-IIM and the positive anti-MDA5-IIM. ResultsCompared with the negative anti-MDA5-IIM patients, the significantly higher incidences of Gottron sign (71.0% vs. 38.3%), ulcerative rash (45.2% vs.16.7%), dyspnea (64.5% vs. 36.7%), and ILD (71.0% vs. 43.3%), in the positive anti-MDA5-IIM patients (P<0.05). Compared with the negative anti-MDA5-IIM patients, the more ground-glass opacification (GGO) in the positive anti-MDA5-IIM patients on high resolution computed tomography (81.8% vs.53.8%, P<0.05). Compared with the positive anti-MDA5-IIM patients without ILD, the positive anti-MDA5-IIM patients with ILD had significantly higher incidence of Gottron sign (77.3% vs. 33.3%), the increased levels of lactate dehydrogenase [(525.20±203.09) IU/L vs. (321.73±188.88) IU/L], Krebs von den Lungen-6 [(1399.55±869.64) U/mL vs. (371.56±128.92) U/mL], and D-dimer [(1226.65±902.31) U/L vs. (703.73±160.11) U/L], as well as higher positive rate of anti-Ro-52 antibody (68.2% vs. 22.2%) and the higher case fatality rate (54.5% vs. 0.0%), with all P<0.05. The triple treatment of high-dose glucocorticoid (GC) combined with intravenous cyclophosphamide (IVCY) and another immunosuppressive agent (tacrolimus or tofacitinib) was found to be effective in the MDA5-IIM associated ILD group, with a lower case fatality rate (28.6%) than high-dose GC combined with IVCY group (47.1%). Conclusions The positive anti-MDA5-IIM with ILD of patients have the higher incidence of rash and dyspnea, with GGO as the most common imaging manifestation. When rapidly progressive interstitial lung disease (RPILD) is combined, the case fatality rate higher. Meanwhile, early intensive immunosuppressive therapy may improve the survival rate of RPILD patients with positive anti-MDA5 antibody.