Pirfenidone ameliorates silica-induced lung inflammation and fibrosis in mice by inhibiting the secretion of interleukin-17A.

Silicosis is a global occupational disease characterized by lung dysfunction, pulmonary inflammation, and fibrosis, for which there is a lack of effective drugs. Pirfenidone has been shown to exert anti-inflammatory and anti-fibrotic properties in the lung. However, whether and how pirfenidone is effective against silicosis remains unknown. Here, we evaluated the efficacy of pirfenidone in the treatment of early and advanced silicosis in an experimental mouse model and explored its potential pharmacological mechanisms. We found that pirfenidone alleviated silica-induced lung dysfunction, secretion of inflammatory cytokines (TNF-α, IL-1ß, IL-6) and deposition of fibrotic proteins (collagen I and fibronectin) in both early and advanced silicosis models. Moreover, we observed that both 100 and 200 mg/kg pirfenidone can effectively treat early-stage silicosis, while 400 mg/kg was recommended for advanced silicosis. Mechanistically, antibody array and bioinformatic analysis showed that the pathways related to IL-17 secretion, including JAK-STAT pathway, Th17 differentiation, and IL-17 pathway, might be involved in the treatment of silicosis by pirfenidone. Further in vivo experiments confirmed that pirfenidone reduced the production of IL-17A induced by silica exposure via inhibiting STAT3 phosphorylation. Neutralizing IL-17A by anti-IL-17A antibody improved lung function and reduced pulmonary inflammation and fibrosis in silicosis animals. Collectively, our study has demonstrated that pirfenidone effectively ameliorated silica-induced lung dysfunction, pulmonary inflammation and fibrosis in mouse models by inhibiting the secretion of IL-17A.

[Research on Life Quality Scale for Patients with Idiopathic Pulmonary Fibrosis].

OBJECTIVE: To develop a life quality scale suitable for idiopathic pulmonary fibrosis (IPF) patients, objectively reflecting its changes. METHODS: Authors first put forward a theoretical structure model of a scale according to patient-reported outcome (PRO) scale formulation principle by combining basic theories of Chinese medicine (CM). Then authors developed an initial scale on the basis of various life quality scales for respiratory disease patients by using structural decision making. Totally 34 patients with confirmed diagnosis of IPF were tested by questionnaire. Items were screened using expert importance scoring method, factor analysis, correlation coefficient method, Cronbach's alpha coefficient method. IPF patient reported outcomes (IPF PRO, IP) were finally defined. RESULTS: A new IP scale was developed covering three areas and 38 items. Pearson correlation coefficient for correlation analysis of clinical symptom scores in ST-George Respiratory Questionnaire and IP scale was 0.828 (P < 0.01). Pearson correlation coefficient for correlation analysis of activity ability scores was 0.929 (P < 0.01). Pearson correlation coefficient for correlation analysis of total scores was 0.862 (P < 0.01). By reliability of IP scale itself (reliability) analysis, Cronbach's alpha coefficient was 0.713. By using factor analysis method for data analysis, KMO statistics was 0.902. CONCLUSION: IP scale fully reflected the connotation of IPF patients' quality of life, so it could be used as CM clinical therapeutic effect evaluation tool.

[Clinicopathological characteristics and prognosis of malignant pleural mesothelioma].

OBJECTIVE: To investigate the clinicopathological characteristics and prognosis of malignant pleural mesothelioma. METHODS: Thirty patients with malignant pleural mesothelioma diagnosed between January 2006 and June 2012 in our hospital were studied retrospectively. Clinical manifestations, radiological characteristics, endoscopic features, histopathology, and survival status were analyzed. RESULTS: There were 15 males and 15 females, with a median age of 58 years. The commonest clinical symptoms were dyspnea on exertion (26 cases), followed by chest pain (15 cases). The main radiological manifestations were small to large amount of pleural effusions (28 cases), often accompanied by pleural thickening and/or pleural nodules.Of the 30 cases, 25 were diagnosed through medical thoracoscopy and 1 through surgical thoracoscopy. Thoracic lesions manifested as nodules of diffuse distribution on the diaphragmatic pleura and parietal pleura.Some pleural surface was covered with lesions like white tiles.Histopathological examination showed epithelial type in 24 cases, sarcomatoid type in 5 and biphasic type in 1 case.Immonohistological examination showed that the positive rates of calretinin, MC, D2∼40 were 27, 25 and 19 cases respectively.Fifteen patients received chemotherapy, 2 underwent pleurectomy, and 8 were treated with best supportive care. Twenty-four patients were followed for 1 month to 6 years, and 6 patients were lost.Overall survival time was 1-54 months. Those who survived longer than 24 months received chemotherapy with pemetrexed and cisplatin/carboplatin or pleurectomy. CONCLUSIONS: Clinical manifestations of malignant pleural mesotheliome were nonspecific, medical thoracoscopy can make early diagnosis. The pathological diagnosis of malignant pleural mesothelioma was based on immunohistochemical examination, calretinin, MC and D2-40 had positive diagnostic value. Malignant pleural mesothelioma had poor prognosis, chemotherapy with pemetrexed and cisplatin/carboplatin could prolong the survival time of the patients.

Expert Consensus on the Diagnosis and Treatment of Anticancer Drug-Induced Interstitial Lung Disease.

Drug-induced interstitial lung disease (DILD) is the most common pulmonary adverse event of anticancer drugs. In recent years, the incidence of anticancer DILD has gradually increased with the rapid development of novel anticancer agents. Due to the diverse clinical manifestations and the lack of specific diagnostic criteria, DILD is difficult to diagnose and may even become fatal if not treated properly. Herein, a multidisciplinary group of experts from oncology, respiratory, imaging, pharmacology, pathology, and radiology departments in China has reached the "expert consensus on the diagnosis and treatment of anticancer DILD" after several rounds of a comprehensive investigation. This consensus aims to improve the awareness of clinicians and provide recommendations for the early screening, diagnosis, and treatment of anticancer DILD. This consensus also emphasizes the importance of multidisciplinary collaboration while managing DILD.

The Potential Diagnostic Biomarkers for the IgG Subclass in Coal Workers' Pneumoconiosis.

Evidence suggests that exposure to coal dust increases immunoglobulin concentration. However, there is a paucity of data reporting immunoglobulin G (IgG) subclass in coal workers' pneumoconiosis (CWP). Therefore, this study intended to evaluate potential diagnostic biomarkers for the disease. CWP patients, dust-exposed workers without pneumoconiosis (DEW), and matched healthy controls (HCs) presented to the General Hospital of Datong Coal Mining Group and Occupational Disease Prevention and Treatment Hospital of Datong Coal Mining Group between May 2019 and September 2019 were recruited. The serum immunoglobulin concentration was determined by the multiplex immunoassay technique. Totally, 104 CWP patients, 109 DEWs, and 74 HCs were enrolled. Serum levels of IgG1, IgG2, IgM, and IgA were elevated in CWPs compared with those in DEWs and HCs (P < 0.05). The order of diagnostic accuracy between CWPs and DEWs depicted by the receiver operating characteristic (ROC) curve was IgG2, IgM, IgG1, IgG3, and IgA. Significantly higher IgG1/IgG3 and IgG2/IgG3 ratios were observed in the CWP group than in DEW and HC groups. Based on the IgG2/IgG3 ratio, the area under the ROC curve between CWP and DEW was 0.785 (95% CI 0.723-0.838), with a sensitivity of 73.1% and a specificity of 73.4%. Our findings suggest that IgG1, IgG2, IgM, and IgA are higher in the CWPs than DEWs and HCs. The IgG2/IgG3 ratio provides a viable alternative for the diagnosis of CWP.

[Effects of aerosolized earthworm fibrinolytic enzyme on bleomycin induced pulmonary fibrosis in rats].

OBJECTIVE: To explore the effects of aerosolized earthworm fibrinolytic enzyme (EFE) on bleomycin-induced pulmonary fibrosis in rats. METHODS: A total of 72 male SD rats were divided randomly into 3 groups of bleomycin (BLM) group with intratracheal BLM (5 mg/kg), control group with the same dose of normal saline, then after both receiving aerosolization of normal saline once daily instead of EFE, EFE group with EFE (2500 U/kg) by aerosolization once daily after BLM instillation. Lung histopathology, immunohistochemistry for transforming growth factor ß(1) (TGF-ß(1)), lung hydroxyproline contents, levels of urokinase PA (uPA), tissue plasminogen activator (tPA) and PA inhibitor 1 (PAI-1) in lung and blood were observed at Days 7, 14 and 28 of experiment, respectively. RESULTS: Compared with BLM group, pulmonary fibrosis improved and the TGF-ß(1) expression of lung tissue decreased (P < 0.01). Hydroxyproline content of lung tissue decreased in EFE group compared with BLM group ((5.8 ± 2.5) vs (9.6 ± 1.3), (6.7 ± 1.4) vs (9.7 ± 1.5), (7.5 ± 1.2) vs (9.7 ± 1.4) mg/L, P < 0.01). Compared with BLM group, the uPA levels of lung were elevated in EFE group at Days 7 and 14 ((1.04 ± 0.36) vs (0.72 ± 0.11), (0.90 ± 0.09) vs (0.75 ± 0.08) µg/L, P < 0.05). Moreover, the plasma levels uPA of increased at Days 14 and 28 ((0.32 ± 0.04) vs (0.25 ± 0.02), (0.36 ± 0.05) vs (0.28 ± 0.04) µg/L, P < 0.05). Consistently, compared with BLM group, the tPA levels of lung increased in EFE group ((4.70 ± 0.87) vs (3.01 ± 0.62), (5.72 ± 0.37) vs (3.00 ± 0.51), (6.73 ± 1.12) vs (3.18 ± 0.38) µg/L, P < 0.01) and the plasma levels of tPA also increased ((3.40 ± 0.36) vs (1.79 ± 0.38), (3.17 ± 0.37) vs (2.18 ± 0.17), (3.85 ± 0.56) vs (2.80 ± 1.06) µg/L, P < 0.01). However, compared with BLM group, the PAI-1 levels of lung decreased in EFE group ((6.04 ± 0.81) vs (8.52 ± 1.01), (6.78 ± 0.81) vs (9.81 ± 1.73), (7.63 + 0.99) vs (11.44 ± 2.54), P < 0.05) and the plasma levels of PAI-1 also decreased in EFE group ((4.82 ± 0.42) vs (6.89 ± 0.84), (5.73 ± 0.40) vs (7.30 ± 1.09), (5.64 ± 0.87) vs (7.98 ± 1.10) µg/L, P < 0.05). CONCLUSIONS: Earthworm fibrinolytic enzyme may decrease bleomycin-induced pulmonary fibrosis and TGF-ß(1) expression while increasing fibrinolytic activation. And fibrinolytic strategies are probably useful for the therapy of fibrotic lung diseases.

[Pulmonary capillary hemangiomatosis: a clinicopathologic analysis of 2 cases with review of literature].

OBJECTIVE: To investigate the clinicopathologic features of pulmonary capillary hemangiomatosis (PCH). METHODS: The clinical and pathologic profiles of 2 PCH cases were evaluated. Immunohistochemical study (EnVision method) was performed on fixed tissues. The biologic behavior was analyzed with follow-up data. RESULTS: The main presenting symptom was dyspnea. Chest radiography of the two cases depicted diffuse, ground-glass nodules, accompanied by enlarged central pulmonary arteries. Microscopically, the most distinctive feature was proliferation of capillary channels within pulmonary interstitium and alveolar walls, accompanied by muscularization of arterioles. Immunohistochemical study showed an abundance of mast cells in the lesion, and staining for platelet-derived growth factor receptor-beta (PDGFR-ß) localized to vascular smooth muscles surrounding the proliferating capillaries and the mast cells. The index of Ki-67 was less than 1 percent and the p53 was negative. CONCLUSIONS: PCH is a rare vascular proliferative disease of yang patients. Increased number of mast cell and the up-regulation of PDGFR-ß may suggest mechanism for PCH. The clinical and radiologic diagnosis of PCH can be very difficult, and the histological examination is regarded as the most reliable means to establish the diagnosis. Pathologists should improve their knowledge on PCH.

Safety and accuracy of transbronchial lung cryobiopsy in diagnosing desquamative interstitial pneumonia.

INTRODUCTION: Transbronchial lung cryobiopsy (TBLC) is a new technique to obtain specimens for diagnosis of interstitial lung disease (ILD) in recent years. The objective of this study is to evaluate the safety and the diagnostic accuracy of TBLC in patients of desquamative interstitial pneumonia (DIP). METHODS: In this study twelve patients confirmed with DIP were selected from January 2019 to December 2020 at the department of pulmonary and critical care medicine in China-Japan Friendship Hospital. All cases underwent TBLC in a hybrid cone beam CT (CBCT) operation room with a single general anesthesia. The definitive diagnosis was made by a multidisciplinary team that involved clinicians, radiologists and pathologists. This study analyzed the biopsy sample surface areas, main complications and the consistency between TBLC pathology and multidisciplinary discussion (MDD) diagnosis for DIP. RESULTS: An average of 3.1 ± 1.1 specimens were obtained per patient. The mean surface area of the specimen was 23.7 ± 6.1 mm2 . None of the cases had pneumothorax or massive hemorrhage. Ten cases (83.3%) had no or mild bleeding and two cases (16.7%) had moderate bleeding. All cases had the typical pathologic characteristics of DIP, which was highly consistent with the diagnosis of MDD. CONCLUSION: TBLC can obtain sufficient samples for the pathological diagnosis of DIP, which has high security and accuracy in experienced specialist centers.

[Quantitative comparison of idiopathic interstitial pneumonias on high-resolution computed tomographic findings].

OBJECTIVE: To analyze the high-resolution computed tomographic (HRCT) findings of IPF (interstitial pulmonary fibrosis), NSIP (nonspecific interstitial pneumonia) and COP (cryptogenic organizing pneumonia) retrospectively through quantification methods and to explore their distinguishing features. METHODS: Observers with no prior knowledge of the diagnosis evaluated the frequency, extent and distribution of various thin-section CT findings in 29 males and 17 females. The mean age was 50 ± 10 years old (range: 25 - 76). They had a histological diagnosis of IPF (n = 19), nonspecific interstitial pneumonia (NSIP) (n = 14) and cryptogenic organizing pneumonia (COP) (n = 13). RESULTS: Ground-glass opacity, thickening of bronchovascular bundles and interlobular septal thickening were frequent features of IPF and NSIP. The frequency and extent of honeycombing and bronchiolectasis were more found in IPF than in NSIP and COP (P < 0.05). The frequency and extent of air space consolidation were more found in COP than IPF (P < 0.05). There were more number of segments with traction bronchiectasis and less extent of air space consolidation in IPF than NSIP and COP. The number of segments with traction bronchiectasis was less in NSIP than that of IPF and COP. CONCLUSION: The various subtypes of idiopathic interstitial pneumonias often have the distinguishing characteristics easily identified on HRCT. Bronchiolectasis and honeycombing are valuable features for IPF; air space consolidation is a valuable feature for COP. The features of NSIP are also found in both IPF and COP so that additional features are required for both.

[Pathologic diagnosis and clinical analysis of chronic extrinsic allergic alveolitis].

OBJECTIVE: To study the clinicopathologic features and diagnostic approach of chronic extrinsic allergic alveolitis (EAA). METHODS: Seven cases of chronic EAA diagnosed by open lung biopsy or lung transplant were enrolled into the study. The clinical and pathologic features were analyzed and the literature was reviewed. RESULTS: There were altogether 4 men and 3 women. The age of the patients ranged from 30 to 65 years (mean = 48 years). All cases represented chronic form and five cases diagnosed by open lung biopsy also showed features of recent aggravation, leading to hospitalization. Four cases had known history of exposure to inciting gases, pollens and pets, and only 2 cases were positive for allergens. High-resolution CT scan showed ground-glass attenuation and reticular pattern that often had a patchy distribution and central predominance. Bronchoalveolar lavage analysis showed marked lymphocytosis, with CD4(+)/CD8(+) ratio less than 1. Lung function test demonstrated a restrictive ventilatory defect, with decreased compliance, reduced diffusion capacity and high airway obstruction. Five cases had open lung biopsy performed and two cases had undergone lung transplantation. Pathologic examination showed bronchiolocentric cellular interstitial pneumonia, interstitial fibrosis, non-caseating epithelioid granulomas, epithelioid histiocytic infiltrate in the respiratory bronchioles and intraluminal budding fibrosis. The five cases with open lung biopsy performed also showed neutrophilic infiltrate in the alveoli. The two lung transplant cases were complicated by severe fibrotic changes. CONCLUSIONS: Chronic EAA demonstrates characteristic pathologic features. Definitive diagnosis requires correlation with clinical and radiologic findings due to possible morphologic mimicry of other diffuse parenchymal lung diseases.

[Tracheobronchomegaly: a report of 3 cases and literature review].

OBJECTIVE: To analyze the clinical, radiological, and pathological characteristics of tracheobronchomegaly (TBM, Mounier-Kuhn syndrome). METHODS: The clinical, radiological and pathological characteristics of 3 cases of TBM were analyzed, and the literatures were reviewed. RESULTS: All 3 patients were men, between the age of 58 - 71 years old. From the onset to diagnosis, the shortest time was 2 months, and the longest 43 years. The most usual presentations included recurrent cough and sputum, and occasional haemoptysis. In the advanced stage of the disease, patients would present shortness of breath and the symptoms associated with respiratory failure because of the reduction in pulmonary function. All the diagnoses were confirmed by X-ray and CT of the chest finding that the trachea and the main bronchi dilated markedly. After anti-infection treatment, all patients recovered. Mounier-Kuhn syndrome was a rare congenital abnormality characterized by atrophy or absence of elastic fibers and thinning of smooth muscle layer in the trachea and main bronchi. These airways were thus flaccid and markedly dilated on inspiration and collapsed on expiration. The usual presentation was recurrent respiratory tract infections with a broad spectrum of functional impairment ranging from minimal disease with preservation of lung function to severe disease in the form of bronchiectasis, emphysema and pulmonary fibrosis, ultimately culminating in respiratory failure and death. Computed tomography scan of the chest was used for the diagnosis. Treatment was mainly supportive with chest physiotherapy and antibiotics. CONCLUSIONS: Mounier-Kunh syndrome should be suspected in patient with recurrent respiratory infections and chronic sputum production. A careful analysis of the central airways at the chest radiograph of these patients is required.

[The relationship between gastroesophageal reflux disease and idiopathic pulmonary interstitial fibrosis].

OBJECTIVE: To determine the prevalence of gastroesophageal reflux disease (GERD) in patients with idiopathic pulmonary interstitial fibrosis (IPIF). METHODS: From December 2006 to January 2008, 24 consecutive patients with IPIF admitted to Beijing Chaoyang Hospital underwent 24-hour esophageal pH monitoring and esophageal manometry. Meanwhile, 23 patients with diffuse parenchymal lung disease (DPLD) (excluding IPIF) admitted to the hospital in the same period served as a control group. Comparison of the prevalence of pathologic esophageal acid exposure GERD symptoms, and ineffective esophageal motility (IEM) between the two groups was made. In this study, nocturnal acid exposure is defined as acid reflux episodes occurring from 10pm to 6am. RESULTS: (1) 16 out of the 24 (66.7%) patients with IPIF were demonstrated to have pathologic esophageal acid exposure; the prevalence of GERD in IPIF patients was significantly higher than that in other DPLD patients, whose prevalence was 26.1% (P < 0.05) ; (2) 87.5% patients with IPIF and GERD (GERD-IPIF) had nocturnal acid exposure episodes; (3) only 37.5% of the GERD-IPIF patients was found to have typical GERD symptoms such as heartburn and regurgitation; (4) The prevalence of IEM was similar in IPIF and other DPLD patients, being 42.9% and 39.1% respectively (P > 0.05). CONCLUSIONS: IPIF patients have higher prevalence of GERD and most of them usually do not show typical reflux symptoms. It is hereby suggested that IPIF patients should be screened with pH monitoring for GERD.

[Chest high resolution CT features of extrinsic allergic alveolitis and its diagnostic value].

OBJECTIVE: To summarize the chest high-resolution CT (HRCT) features of the patients with extrinsic allergic alveolitis (EAA). METHODS: We analyzed the images of chest HRCT of 34 patients diagnosed as EAA at our hospital from February 2001 to August 2009. RESULTS: All patients had a history of environmental exposure. The duration of intermittent or continuous antigen exposure was from 3 months to 13 years. Two patients showed acute clinical manifestations. There were 22 sub-acute and 10 chronic cases. Acute EAA was characterized by ground-glass opacities, air trapping and/or mosaic sign on HRCT. The HRCT features of subacute EAA included patchy ground-glass opacities with mosaic sign (n = 11, 50.0%) and diffusely distributed centrilobular nodules (n = 7, 31.8%) with mosaic sign (n = 4, 18.2%). All patients with chronic EAA had reticular and honeycombing lesions on HRCT. There were 3 cases with ground-glass opacities, 3 with mosaic sign, and 3 with centrilobular nodules. CONCLUSION: The typical findings of chest HRCT are helpful for making a diagnosis and differential diagnosis of EAA.

[Causes of respiratory failure complicating interstitial lung disease and application value of mechanical ventilation].

OBJECTIVE: To investigate the potential causes of respiratory failure in patients with interstitial lung disease (ILD) and evaluate the application value of mechanical ventilation (MV) in its treatment. METHODS: This study included the clinical data of 47 ILD patients (29 males and 18 females) complicating respiratory failure and admitted to respiratory care unit (RICU) for receiving MV at Beijing Chaoyang Hospital from January 1, 1998 to June 30, 2008. The median age was 67 years old. And a retrospective analysis was conducted for clinical characteristics, potential causes of respiratory failure, RICU treatment, prognosis and causes of death, etc. RESULTS: The causes of respiratory failure were as follows: respiratory infections (n = 18, 38.3%), respiratory infection superimposed aggravated ILD (n = 15, 31.9%), pulmonary embolism (n = 8, 17.0%), ILD with acute exacerbation (n = 5, 10.6%) and heart failure (n = 1, 2.1%). And the mortality rates of RICU were 55.6% (10/18), 73.3% (11/15), 5/8, 4/5 and 1/1 respectively. Upon initial admission into RICU, 31 cases received noninvasive ventilation and 16 cases tracheal intubation. The mortality rates were 54.8% and 87.5% respectively. And there was statistically significant difference between the groups (chi(2) = 5.014, P = 0.025). In all patients, the RICU mortality rate was 66.0% (31/47) and the hospital mortality rate 70.2% (33/47). CONCLUSIONS: Respiratory infection, pulmonary embolism and acute exacerbation of ILD are the main common causes of respiratory failure in ILD patients. Noninvasive ventilation therapy is clinically preferable to invasive ventilation for ILD patients with respiratory failure.

Water-Soluble C60 Protects Against Bleomycin-Induced Pulmonary Fibrosis in Mice.

BACKGROUND: Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic interstitial pneumonia. And, oxidation/antioxidant imbalance plays an important role in the progress of IPF. Fullerene is considered to be a novel "structural" antioxidant. This study aimed to explore if water-soluble C60 (C60(OH)22) can exhibit antifibrotic activity in its antioxidant role. METHODS: Healthy C57BL/6J mice were randomly grouped and induced pulmonary fibrosis by intratracheal injection of bleomycin. RESULTS: The survival rate of mice was observed and found that 10mg/kg was the optimal dose of water-soluble C60 for pulmonary fibrosis. We observed that water-soluble C60 can alleviate the severity of pulmonary fibrosis by observing the chest computed tomography, pulmonary pathology, and content of collagen, alpha smooth muscle actin and fibronectin in lung. Compared with bleomycin group, ROS, the content of TNF-α in BALF, and the number of fibroblasts was significantly decreased and the number of type Ⅱ alveolar epithelial cells was increased after treatment with C60. CONCLUSION: Therefore, thanks to its powerful antioxidant action, water-soluble C60 can reduce the severity of pulmonary fibrosis induced by bleomycin in mice.

[Association of ENA-78, IP-10 and VEGF gene polymorphism with idiopathic pulmonary fibrosis].

OBJECTIVE: To examine whether there was an association between epithelial neutrophil activating peptide 78 (ENA-78), interferon-inducible protein 10 (IP-10), vascular endothelial growth factor (VEGF) polymorphism and Chinese patients with idiopathic pulmonary fibrosis (IPF). METHODS: Polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) were performed to analyze the gene polymorphisms of ENA-78 (-156G/C), IP-10 (-1596C/T) and VEGF (+405G/C) in 60 IPF patients and 60 controls with trauma or bone fracture matched in age, gender and smoking status. RESULTS: The distribution of carrying GC + CC genotype frequency (20.0%) and C allele frequency (12.7%) for ENA-78 in IPF patients was significantly higher than that in healthy controls [6.7% (P = 0.032) and 3.3% (P = 0.008), respectively], the relative risk of suffering from IPF of -156C allele gene carrier significantly increased (OR = 4.23, 95%CI: 1.35-13.20). The distribution of carrying CT + TT genotype frequency (10.0%) and T allele frequency (5.8%) for IP-10 in IPF patients was significantly lower than that in healthy controls [26.7% (P = 0.018) and 14.2% (P = 0.031), respectively], the relative risk of suffering from IPF of -1596T allele gene carrier decreased (OR = 0.38, 95%CI: 0.15-0.95). No association was found between VEGF (+405G/C) polymorphism and IPF. CONCLUSIONS: -156C allele for ENA-78 may be a risk factor of IPF and -1596T allele for IP-10 a beneficial factor of IPF. The VEGF (+405G/C) gene polymorphism has no effect upon the predisposition to IPF.

[A questionnaire survey on the current practices of respiratory care in intensive care unit in 30 provinces].

OBJECTIVE: To investigate respiratory care equipment, operators and conditions of performance in intensive care units (ICU), with the aim of providing data for standardization and developing respiratory care in China. METHODS: A questionnaire survey was performed in one national and two international conferences in August, 2006. Four hundred and ninety-one doctors and nurses from 320 ICUs in 264 tertiary hospitals responded. RESULTS: Ratios of invasive and noninvasive mechanical ventilators to beds were 0.52:1 (2 189/4 185) and 0.16:1 (672/4 185), respectively. Of 320 ICUs, ratios of ICU equipped with ultrasound, jet nebulizers and MDI were 55.9% (179/320), 33.8% (108/320) and 12.1% (39/320), respectively, and percentages of doctors in charge of setting modes and parameters, weaning and extubation were 92.1%, 93.1%, 83.5%, respectively. Suction (93.9%), humidification (90.2%), aerosol therapy (91.6%) and circuit changing (83.7%) were nurses' duties. Among 491 responders, 40.9% of them implemented spontaneous breathing trials (SBT) before weaning, 13.4% were ignorant of it, and 12.8% never. 27.1% of ICU never monitored air temperature during invasive mechanical ventilation, 34.4% provided humidification by instilling or pumping saline continuously for those patients who were weaned from ventilators but not extubated, 55.6% checked ventilator before use. Ventilator circuits were changed once a week in 48.1%, 1-3 days in 25.0% and 3-5 days in 14.7%. CONCLUSION: The quantity of ventilators in the ICU has increased, but other practical respiratory care equipment have not been used widely. Most of respiratory care services are still provided by nurses and doctors, lacking professional staffs. The management is evidently variable but without a standardized guideline.

Obstructive Sleep Apnea in Patients With Fibrotic Interstitial Lung Disease and COPD.

STUDY OBJECTIVES: Despite the clinical and prognostic significance of obstructive sleep apnea (OSA) in chronic respiratory diseases (CRDs), there have been few studies about the possible predictors of OSA and the effect of OSA on quality of life in patients with CRDs. The objectives were to identify physiological and clinical parameters that predict the occurrence and severity of OSA and to investigate the effect of OSA on quality of life in patients with CRDs. METHODS: Seventy-three patients with chronic obstructive pulmonary disease (COPD) and 77 patients with fibrotic interstitial lung disease (ILD) underwent overnight polysomnography (PSG) and pulmonary function testing and completed clinical questionnaires. The oximetry tracing was interpreted blindly with respect to the PSG results. RESULTS: The prevalence of OSA was 44% and 62% in COPD and ILD, respectively. The COPD assessment test item scores related to sleep quality and daily vitality were worse among patients with OSA than among patients without OSA. The STOP-BANG questionnaire (cutoff point ≥ 3) and oxygen desaturation index from the oximetry recording (oxygen desaturation index (ODI) were associated with OSA in CRDs. The STOP-BANG questionnaire with a cutoff point ≥ 3 or 6 had the highest sensitivity and specificity, respectively, in detecting OSA in CRDs. ODI had the best accuracy in identifying OSA and was independently associated with the apnea-hypopnea index in CRDs. CONCLUSIONS: We found OSA to be common and associated with worse sleep quality and less daily vitality in patients with advanced CRDs. The STOP-BANG questionnaire with different cutoff points may help rule in or rule out OSA. Overnight oximetry can be used as a screening tool for OSA and can assist the clinical evaluation of OSA in patients with CRDs.

[Clinical features of adult pulmonary Langerhans' cell histiocytosis].

OBJECTIVE: To investigate the clinical features including history, radiology, pulmonary function and histological pathology of adult pulmonary Langerhans' cell histiocytosis (PLCH). METHOD: Clinical data of 5 PLCH patients were reviewed from June 2006 to October 2007. RESULTS: All 5 patients were smokers with lung involvements. Spontaneous pneumothorax was the initial manifestation in 2 patients. One end-stage patient presented with severe pulmonary arterial hypertension. High-resolution CT scans of the lungs showed multifocal nodules and cysts that predominantly affected the upper and middle lung zones. At microscopic analysis, lung specimen showed discrete bronchiolocentric stellate lesions separated by uninvolved lung with air-space enlargement. Langerhans' cells expressed cell surface CD1a and cytoplasmic S-100 protein. Cessation of smoking led to stabilization of the disease. The decrease of V50 (53.6%-77.6% pred) and V25 (38.5%-70.5% pred) was consistent with small airway damages of lung histology. CONCLUSION: PLCH is an isolated form of Langerhans' cell histiocytosis that primarily affects a single-system in smokers.