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.
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.
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.
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 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.
ObjectiveTo improve clinicians' knowledge of hypersensitivity pneumonitis (HP). MethodsWe retrospectively analyzed the clinical data of 24 HP patients who were diagnosed in the Affiliated Drum Tower Hospital of Nanjing University Medical School during February 2005 to February 2013. The clinical,radiological and pathological features of those patients were summarized. ResultsAmong those 24 patients,15 were male and 9 were female,with mean age of (48±13) years. All patients had a history of environmental exposure. Two patients showed acute clinical manifestations,and there were 17 subacute and 5 chronic cases. The main clinical manifestations were dyspnea,cough,sputum,fever and weight loss with hypoxemia via blood gas analysis. Restrictive ventilatory impairment was the most frequent functional pattern,and the carbon monoxide diffusing capacity was decreased. Pulmonary function test showed restrictive ventilatory defect and gas interchange disturbance. The features of chest HRCT included diffuse ground-glass attenuation and/or patchy consolidation,centrilobular micronodules,mosaic sign,reticular and/or honeycombing lesions. Bronchoalveolar lavage fluid (BALF) demonstrated an increase of total cell counts with predominant lymphocytosis. The transbronchoscopic lung biopsy (TBLB) pathological examination revealed lymphocytic alveolitis,noncaseating granuloma,and interstial pneumonia. All patients were treated by corticosteroid and avoided antigen exposure and showed significant clinical and radiological improvement. ConclusionThe diagnosis of HP is difficult. In most cases (acute and subacute HP),a diagnosis can be made by combination history of exposure,chest HRCT manifestations,cell classification of BALF and pathological examination of TBLB. For atypical cases (chronic HP),a surgery lung biopsy is needed for multi-disciplinary diagnosis including pathologist,radiologist and pulmonologists.
Objective To analyze the clinical symptoms, imaging, laboratory tests, efficacy and other indicators of patients diagnosed as diffuse parenchymal lung disease ( DPLD) , in order to provide a reference for differential diagnosis of secondary DPLD and idiopathic interstitial pneumonia ( IIP) .Methods Sixtytwo patients diagnosed as DPLD were retrospectively analyzed. Results In 62 patients with DPLD, 19 patients ( 30. 6% ) were diagnosed as secondary DPLD, 42 cases ( 67. 7% ) as IIP, 1 case ( 1. 6% ) as Langerhans cell histiocytosis. The smoking rate of the DPLD patient was 33. 8% , which was higher than general population ( 29. 7% ) . 94. 7% of the secondary DPLD patients had cough, which was higher than the IIP patients ( 71. 4%) . The average age of onset of the secondary DPLD and IIP was ( 45. 9 ±16. 8) years and ( 60. 5 ±7. 7) years respectively, without significant difference ( P gt; 0. 05) . Etiological factors of secondary DPLD were dust, pets, drugs, pesticides, decoration material, etc. The secondary DPLD patients had higher response rate to steroid therapy, but had no statistical difference compared with the IIP patients ( 46. 2% vs. 37. 5% , P gt;0. 05) . Conclusions As a group of diseases of known causes, history taking is very important for DPLD diagnosis and differential diagnosis. Clinical symptoms, imaging, and laboratory tests may provide reference for differential diagnosis of secondary DPLD and IIP.
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.