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.
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 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 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 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 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.
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.
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 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 effect of prone position ventilation (PPV) on hemodynamics in patients with interstitial lung disease (ILD) concurrent with acute respiratory distress syndrome (ARDS) or not. Methods Severe ARDS patients received PPV treatment in intensive care unit from 2013 to 2015 were retrospectively analyzed. Pulse index continuous cardiac output (PiCCO) monitoring indices were compared 2h before PPV (PPV-2), in-PPV (PPVmean) and 2h after turn back to supine position (PPV+2), including mean arterial pressure (MAP), cardiac index (CI), intrathoracic blood volume index (ITBVI), etc. Heart function, liver function, renal function, coagulative function were compared before and after all the PPV treatment. Results Thirty-six severe pulmonary ARDS patients were recruited. Norepinephrine (NE) dose was (0.14±0.10) μg·kg-1·min-1 and lactate concentration was (1.70±0.57) mmol/L before PPV. CI in all the patients was increased [(4.06±0.95) L·min-1·m-2 vs. (3.98±1.05) L·min-1·m-2, P > 0.05) and central venous oxygen saturation, ITBVI [(76.94±8.94)% vs. (70.67±8.23)%, (982.17±245.87) mL/m2 vs. (912.97±177.65) mL/m2, P < 0.05)] were significantly increased compared with PPV-2, while heart rate (HR), MAP, urine volume were decreased (P > 0.05). The patients were divided into an ILD group (n=17) andanon-ILD group (n=19),and no significant differences were found in baseline data between two groups. Compared with PPV-2, MAP was significant decreased at PPV+2 [(85.44±10.84) mm Hg vs. (89.21±10.92) mm Hg, P < 0.05) in the ILD group. Compared with PPVmean, CI was obviously declined at PPV+2 in the ILD group. Activated partial thromboplastin time was increased [(67.04±97.52)s vs. (41.24±8.72) s, P < 0.05] and BUN was higher [(10.64±4.95) μmol/L vs. (8.18±3.88) μmol/L, P < 0.05] in the ILD group, while no significant difference was found in the non-ILD group. Conclusion PPV will not affect cardiac output in severe ARDS patients with ILD. It can increase venous return and improve tissue perfusion, but the perfusion of the abdomen organ need to be monitored cautiously.