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find Keyword "特发性肺纤维化" 25 results
  • Acute Exacerbation of Idiopathic Pulmonary Fibrosis: Clinical Analysis of 21 Cases

    Objective To improve the awareness of acute exacerbation of idiopathic pulmonary fibrosis ( AEIPF) and discuss its clinical characteristics, diagnosis, treatment and outcome. Methods The clinical data of patients with AEIPF from June 2006 to June 2011 in 11 hospitals in Jiangsu were collected and analyzed. Resluts There were 18 males and 3 females in the AEIPF patients with mean age of ( 67.4 ± 8.1) years. The duration from IPF diagnosis was ( 7.4 ±8.2) months. The duration of acute symptom before admission was ( 7.0 ±5.3) days. The distribution pattern of new groud-glass opacity was peripheral in 3 patients,multifocal in 5 patients, and diffuse in13 patients. All patients were treated with corticosteroid pulse therapy. Nine patients survived and 12 patients died. The mortality rate was 57.1% . Conclusions AEIPF progresses quickly and the mortality rate is very high. Corticosteroid pulse therapy is the mainstay of therapy in AEIPF patients.

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  • Clinicoradiologic Features of Acute Exacerbation of Idiopathic Pulmonary Fibrosis: Two Cases Report and Literature Review

    Objective To analyze the clinical presentations and radiological characteristics of acute exacerbation of idiopathic pulmonary fibrosis ( IPF) . Methods Clinical and radiological data of 2 patients with acute exacerbation of IPF from April 2006 to July 2008 were retrospectively analyzed and literatures were reviewed. Results Both patients were senior male patients over 60 years old. Dyspnea, cough and inspiratory crackles were the major symptoms and signs. Two patients were experiencing an exacerbation of dyspnea for one week and half of month, respectively. PaO2 /FiO2 of both patients was less than225 mm Hg. In both patients, high-resolution computed tomography ( HRCT) scans at the exacerbation showed typical signs of IPF including peripheral predominant, basal predominant reticular abnormality, with honeycombing and traction bronchiectasis and bronchiolectasis, and newly developing alveolar opacity. HRCT scan showed peripheral area of ground-glass attenuation adjacent to subpleural honeycombing in one patient, and diffusely distributed ground-glass opacity in another patient. Two patients had received corticosteroid treatment. For one patient, the symptoms improved, and ground-glass attenuation adjacent to subpleural honeycombing had almostly resolved. The other patient died of respiratory failure. Conclusions Some acute exacerbation in idiopatic pulmonary fibrosis can be idiopathic. The clinical presentations mainly include the worsening of dyspnea within short time. HRCT generally demonstrates new bilateral ground-glass abnormality with or without areas of consolidation, superimposed on typical changes of IPF.

    Release date:2016-09-14 11:23 Export PDF Favorites Scan
  • Sleep Apnea in Idiopathic Pulmonary Fibrosis

    Objective To investigate the prevalence of obstructive sleep apnea hypopnea syndrome ( OSAHS) in patients with idiopathic pulmonary fibrosis ( IPF) and its clinical significance. Methods Sleep quality and breathing disorders were measured by polysomnography and the relationship with lung function was analyzed in 20 IPF patients. Results Thirteen of 20 subjects ( 65% ) had OSAHS as defined by an AHI ≥5 events per hour. Three subjects ( 15% ) had mild OSAHS ( AHI,5 to 20 events per hour) , and 10 subjects ( 50% ) had moderate-to-severe OSAHS ( AHI≥20 events per hour) . The sleep architecture in these patients showed a reduction in sleep efficiency, rapid eye movement ( REM) sleep and slow wave sleep, and a marked sleep fragmentation due to an increased arousal index. The AHI was negatively correlated with FVC% pred ( r =-0.672, P=0.001) and FEV1% pred ( r =-0.659, P=0.002) , and positively correlated with body mass index ( BMI) ( r=0.791, Plt;0.0001) . Conclusions OSAHS is a common comorbidity in IPF. Early treatment of OSAHS may improve quality of life and the prognosis of patients with IPF.

    Release date:2016-08-30 11:56 Export PDF Favorites Scan
  • Causal relationship between gut microbiota and idiopathic pulmonary fibrosis: A bi-directional two-sample Mendelian randomization study

    ObjectiveTo investigate the causal relationship between gut microbiota and idiopathic pulmonary fibrosis (IPF). MethodsGenome-wide association studies (GWAS) data of gut microbiota and IPF were obtained from MiBioGen and Finngen databases, respectively. Instrumental variables were screened by means of significance, linkage disequilibrium, weak instrumental variable screening, and removal of confounding factors (genetics, smoking, host characteristics). Inverse variance weighted (IVW) was used as the main Mendelian randomization (MR) analysis method, and the weighted median, simple mode, MR-Egger, and weighted mode were used to perform MR to reveal the causal effect of gut microbiota and IPF. The Cochrane's Q, leave-one-out, MR-Egger-intercept, and Mendelian randomization pleiotropy residual sum and outlier (MR-PRESSO) and Steiger tests were used to analyze the heterogeneity, horizontal pleiotropy, outliers, and directionality, respectively. ResultsIVW analysis results showed that Actinomycetes [OR=1.773, 95%CI (1.323, 2.377), P<0.001], Erysipelatoclostridium [OR=2.077, 95%CI (1.107, 3.896), P=0.023], and Streptococcus [OR=1.35, 95%CI (1.100, 1.657), P=0.004] could increase the risk of IPF. Bifidobacterium [OR=0.668, 95%CI (0.620, 0.720), P<0.001], Ruminococcus [OR=0.434, 95%CI (0.222,0.848), P=0.015], and Tyzzerella [OR=0.479, 95%CI (0.304, 0.755), P=0.001] could reduce the risk of IPF. No significant heterogeneity, horizontal pleiotropy, outliers, and reverse causality were found. ConclusionActinobacteria, Erysipelatoclostridium and Streptococcus may increase the risk of IPF, while Bifidobacterium, Ruminococcus and Tyzzerella may reduce the risk of IPF. Regulation of the above gut microbiota may become a new direction in the study of the pathogenesis of IPF.

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  • 吡非尼酮治疗特发性肺纤维化: 我们拥有多少证据?

    特发性肺纤维化( idiopathic pulmonary fibrosis, IPF) 为原因不明, 局限于肺, 以普通型间质性肺炎( usual interstitialpneumonitis, UIP) 为病理特征的慢性进行性发展的纤维化性间质性肺疾病。该病预后不良, 现无有效治疗药物[1] , 寻找有效治疗IPF的药物始终是该研究领域的热点之一。迄今已有诸多针对肺纤维化发病机制的基础研究成果, 并陆续转化为新的研究药物[2] 。IPF 临床中心协作网的建立使数以百计的IPF患者比较快地入组参加新药物临床试验, 并顺利完成了多项高质量的双盲、随机、安慰剂对照临床试验, 有力推动了IPF 治疗领域的进展。遗憾的是, 近几年已发表的多项临床试验结果[3-6] 令人失望。在挫折和期盼之中, 最新发表的吡非尼酮( pirfenidone) 治疗IPF临床试验结果[7] 为无药可治的IPF患者带来了一线希望的曙光。

    Release date:2016-08-30 11:53 Export PDF Favorites Scan
  • Developments in researches on acute exacerbation of idiopathic pulmonary fibrosis

    Acute exacerbation of idiopathic pulmonary fibrosis (AE-IPF) is defined as an acute and clinically significant respiratory deterioration characterized by evidence of new, widespread alveolar abnormality. In the past, AE-IPF was considered to be idiopathic, which was hard to be prevented and its prognosis was hard to be obviously improved; the latest researches have shown that AE-IPF can be triggered by known causes, including pulmonary infection, aspiration, etc. This review summarizes the etiology or risk factors, treatment and prevention of AE-IPF according to the latest researches.

    Release date:2018-01-23 02:34 Export PDF Favorites Scan
  • Expression and Significance of KL-6 in BALF and Serum of Patients with Idiopathic Pulmonary Fibrosis

    ObjectiveTo detect the levels of Krebs von den lungen 6 (KL-6) in bronchoalveolar lavage fluid (BALF) and serum of patients with idiopathic pulmonary fibrosis (IPF),and explore its clinical significance. MethodsThirty-four patients with IPF and 10 patients with sarcoidosis in Ⅰ period were recruited in the study. ELISA was used to detect the level of KL-6 in BALF and serum. ResultsIn the IPF group,the forced vital capacity as percentage of predicted value (FVC% pred) and diffusion capacity for carbon monoxide as percentage of predicted value (DLCO %pred) were both significantly lower than those of the sarcoidosis group[(69.51±13.65)% vs. (82.06±5.84)%,(48.58±12.73)% vs. (81.47±6.39)%,P<0.01]. In the BALF of IPF group,the percentage of neutrophils was higher[(8.91±6.79)% vs. (5.50±3.60)%,P<0.05],and the percentages of lymphocytes and CD4/CD8 ratio were lower than those of the sarcoidosis group[(11.71±6.64)% vs. (23.30±12.68)%,(1.46±0.83) vs. (4.01±5.10),P<0.05]. In the IPF group,the level of KL-6 in the BALF and serum was higher than that of the arcoidosis group[(437.43±251.70) U/mL vs. (221.59±127.41) U/mL,(857.81±515.53) U/mL vs. (338.67±168.13) U/mL,P<0.001]. There was obvious correlation between the level of serum KL-6 with FVC%pred and DLCO%pred in the IPF group (r=-0.46,r=-0.58,P<0.05). ConclusionsThe level of KL-6 in BALF and serum is elevated in patients with IPF. There is obvious correlation between the level of serum KL-6 with FVC%pred and DLCO%pred in IPF patients. KL-6 may be an indicator of IPF in clinical diagnose.

    Release date:2016-10-02 04:55 Export PDF Favorites Scan
  • Efficacy of N-acetylcysteine for Patients with Idiopathic Pulmonary Fibrosis: A Meta-Analysis

    ObjectiveTo systematically review the efficacy and safety of N-acetylcysteine (NAC) for patients with idiopathic pulmonary fibrosis (IPF). MethodsWe electronically searched PubMed, EMbase, The Cochrane Library (Issue 2, 2014), CNKI, WanFang Data and VIP databases from the date of establishment to February 2014 for all randomized controlled trials (RCTs) on the use of NAC in patients with IPF. Manual search in relevant journals were also performed. The data extraction and quality assessment of included RCTs were conducted by two reviewers independently. Then, meta-analysis was conducted using RevMan 5.1 software. ResultsA total of 13 RCTs involving 713 patients were included. The results of meta-analysis indicated that the NAC group was better than the control group in clinical effectiveness (RR=1.34, 95%CI 1.19 to 1.51, P < 0.000 1). After treatment, the lung function was also improved in the NAC group than in the control group in the following index:PaO2 (MD=6.06, 95%CI 3.79 to 8.32, P < 0.000 01), vital capacity (VC) (%) (MD=4.79, 95%CI 0.35 to 9.24, P=0.03) and diffusing capacity of carbon monoxide (Dlco) (%) (MD=5.74, 95%CI 2.67 to 8.81, P=0.000 2). However, no significant difference was found between groups in total lung capacity (TLC) (%) (MD=5.56, 95%CI-1.73 to 12.86, P=0.14). No serious or frequently-happened adverse effect was reported in the NAC group. ConclusionThe current evidence suggests that NAC in long term use could improve clinical conditions, PaO2 and lung function of IPF patients, with less adverse effects.

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  • Clinical Analysis on Interstitial Pneumonia with Positive Myeloperoxidase

    Objective To summarize the clinical characteristics of patients with interstitial pneumonia with positive myeloperoxidase (MPO-IP). Methods The clinical data of 15 patients hospitalized with MPO-IP from June 2013 to January 2016 were analyzed retrospectively, including clinical manifestations, laboratory test, lung function test, chest high resolution computed tomography (HRCT) and management. Simultaneously, 11 patients with IPF were recruited as control. Results The onset age of MPO-IP was older than that in IPF patients (74.07±6.31 yearsvs. 66.73±6.80 years,P<0.01). There was no manifestation of vasculitis in kidney in all included patients, and the differences were not significant in gender, smoking, cough and expectoration, wheezing, shortness of breath, HRCT manifestations, FVC%pred, TLCO%pred, PaO2 or PaCO2 between the two groups. In 15 MPO-IP patients, HRCT revealed 12 cases of usual interstitial pneumonitis (UIP), 2 cases of nonspecific interstitial pneumonia (NSIP), 1 case of organizing pneumonia (OP), and 5 patients with overlapped emphysema. Eleven IPF patients were all in UIP pattern and 4 with overlapped emphysema. One MPO-IP patients and 5 IPF patients had clubbed-fingers. The differences in clubbed-finger and treatment were significantly different between the two groups. Nine IPF patients received symptomatic treatment and7 MPO-IP patients were administered with glucocorticoids and immunosuppressants. In addition 2 MPO-IP patients were treated with pirfenidone. Conclusions MPO-IP patients have older onset age of disease and lower incidence of clubbed-finger than IPF patients. UIP is the main HRCT change both in MPO-IP and IPF. Beside glucocorticoid and immunosuppressants, pirfenidone is a choice and worthy of further research in management of MPO-IP.

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  • Guidelines for diagnosis and treatment of Chinese medicine on idiopathic pulmonary fibrosis

    Idiopathic pulmonary fibrosis (IPF) is a chronic fibrotic and interstitial lung disease of unknown cause. It has a serious impact on people's health. Traditional Chinese medicine (TCM) has certain advantages in diagnosing and treating on IPF, which have been widely used in clinic. In order to improve the diagnostic and treatment level for IPF with TCM. The Internal Medicine Committee of World Federation of Chinese Medicine Societies organized and established a multidisciplinary background working group. The document was formulated by referring to the formulation method and process of clinical practice guidelines, which are based on the best evidence and the opinions of clinical physicians and patients. Physicians can use this guideline to make clinical decisions.

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