特发性肺间质纤维化

特发性肺间质纤维化(Idiopathic Puimonary Fibrosis IPF)系指原因不明局限于肺部的间质纤维化,起始表现为弥漫性肺泡炎,继之形成肺间质的大量纤维结缔组织和肺结构紊乱。本病有多个名称,近年已渐一致,认为定名为特发性肺间纤维化较为恰当,是遍及全世界的常见病,绝大多数为慢性型。发病机制一、形态发生机制:肺泡上皮受损后肺泡隔内的成纤维细胞(Fb)、肌纤维细胞、炎症细胞在趋化因子的诱导下伸展到肺泡腔。前两种细胞活化、增殖,产生胶原蛋白。肺泡渗出物粘附于肺泡基底膜,由增殖的Ⅱ型肺泡细胞复盖。表面活性物质丧失后,肺泡萎陷,肺泡壁靠拢。     

特发性肺间质纤维化的研究进展

特发性肺间质纤维化至今病因不明,在诊断和治疗上较为混乱。全球有关专家就此问题展开讨论,达成以下共识。     

016 特发性肺间质纤维化的研究进展

特发性肺间质纤维化至今病因不明,在诊断和治疗上较为混乱.全球有关专家就此问题展开讨论,达成以下共识.     

特发性肺间质纤维化的相关研究进展

特发性间质性肺炎（IIP）是一组原因不明的间质性肺疾病（ILD）,以弥漫性肺泡炎和肺泡结构紊乱最终导致肺纤维化为特征[1].特发性肺间质纤维化（IPF）是IIP中最常见的一种,由于其发病机制复杂,早期诊断困难,病情呈不可逆进展,且易合并其他疾病,目前尚无有效治疗措施,预后极差,是目前国内外较关注的疾病之一,也是研究热点及难点之一.现就近几年IPF的相关研究作一综述.     

特发性弥漫性肺间质纤维化10例分析

作者收治10例I P F患者,均有进行性呼吸困难.7例可闻及Velcro啰音,5例血清粘蛋白升高.X线均见两肺呈弥漫性网状或网结节状阴影,以中下肺野为主.纤维支气管镜活检和支气管灌洗术的结果提示I PF是以中性粒细胞增高为特征的肺泡炎.     

特发性肺间质纤维化的研究进展

特发性肺间质纤维化至今病因不明，在诊断和治疗上较为混乱，全球有关专家就此问题展开讨论，达成以下共识。     

外周血单核细胞DcR3基因过度表达在间质性肺疾病发病中的意义

间质性肺疾病 (ILD)是一组病因复杂、发病机制尚不十分明确的疾病 ,大量资料表明 ,ILD发病与免疫功能紊乱有关 ,Fas/FasL系统介导的凋亡障碍在某些免疫性疾病发病中起重要作用[1] ,但其作用机制尚不清楚 ,对此调节系统方面的研究报道很少。最近发现一个可溶性受体蛋白 诱导受体 3(DcR3) ,能竞争性与FasL结合 ,从而阻断FasL诱导的细胞凋亡 ,使Fas/FasL功能失调 ,在自身免疫性疾病及ILD发病中起一定作用[2 ,3 ] 。我们的实验采用逆转录 多聚酶链反应 (RT PCR)法在国内首次检测DcR3基因在ILD患者及健康人外周血单核细胞 (PBMC)…     

特发性肺间质纤维化的回顾和展望

自50多年前Hamman和Rich报道4例死于原因不明的弥漫性肺间质纤维化以来,特发性肺间质纤维化（idiopathic pulmonary fibrosis,IPF）的概念和定义有了突飞猛进的发展,尤其是近5年,这反映了广大的科研和临床工作者对此类疾病的重视和不断探索。     

乙酰半胱氨酸治疗特发性肺间质纤维化的进展

特发性肺间质纤维化是一种以纤维化及肺实质重塑为特征的渐进性疾病,目前尚无特效疗法,标准疗法是美国胸科协会推荐的治疗方案--皮质激素加免疫抑制剂.然而,尚无充分证据证明其有效性,且由于其不良反应大,常常限制了临床应用.近年来,大剂量乙酰半胱氨酸(600 mg,3次/d)的使用为特发性肺间质纤维化的治疗带来了新的曙光,其具体机制包括:抗氧化,增加细胞内还原型谷胱甘肽;抗凋亡,保护肺泡上皮;抑制成纤维细胞的增殖及胶原合成等.现就近年的研究进展进行综述.     

特发性肺间质纤维化的现代治疗研究进展

特发性肺间质纤维化是一种至今病因及发病机制尚不清楚的疾病，在诊断，治疗等方面还存在很多问题。以下将就近来的研究进展及治疗现状作一概述。     

DcR3基因与特发性肺纤维化肺功能的相关性研究

目的　探讨外周血单核细胞中DcR3基因表达水平与特发性肺纤维化 (IPF)患者的肺限制性通气功能、弥散功能的关系 ,以及IPF的发病机理、DcR3基因在IPF疾病发生、发展过程中的作用机制。方法　应用逆转录聚合酶链反应法 (RT PCR)检测IPF患者外周血单核细胞中DcR3基因表达水平。结果　DcR3基因在IPF患者PBMC中表达 (0 357± 0 0 88)明显高于健康对照组 (0 2 59± 0 0 84) (P <0 0 5)。IPF患者PBMC中DcR3基因表达增高者其肺限制性通气功能障碍和弥散功能障碍均加重。结论　DcR3基因在IPF患者PBMC中表达明显高于健康人 ,DcR3基因表达水平与IPF患者肺限制性通气功能障碍、弥散功能障碍呈正相关。     

Perceptions of fatigue in patients with idiopathic pulmonary fibrosis or sarcoidosis

BackgroundFatigue is highly prevalent in patients with idiopathic pulmonary fibrosis (IPF) or sarcoidosis. However, the difference in fatigue perceptions for these patients is unknown and this may be important to better understand what fatigue means to the individual patient.MethodsThis cross-sectional quantitative study aims to determine the different perceptions of fatigue as ‘frustrating’, ’exhausting’, ‘pleasant’, ‘frightening’ using the Fatigue Quality List and to assess determinants related to these perceptions of fatigue. Beside the fatigue quality connotations, demographics, lung function, fatigue severity (Checklist Individual Strength subscale Fatigue), dyspnea (modified-Medical Research Council), fatigue catastrophizing (Fatigue Catastrophizing Scale), anxiety/depression (Hospital Anxiety and Depression Scale) and general health status (EuroQoL 5-dimension 5-level) were assessed.ResultsMean frequency score of fatigue-related perceptions in patients with IPF was 3.4 points and in patients with sarcoidosis 4.0 points. Severely fatigued patients with IPF reported their fatigue less ‘pleasant’ significantly more often than patients without severe fatigue. Fatigue severity, dyspnea, catastrophizing and general health were significantly correlated with the negative connotation categories of the Fatigue Quality List in patients with IPF. Severely fatigued sarcoidosis patients reported their fatigue perceptions significantly more often as ‘frustrating’, ’exhausting’, ‘frightening’ and less ‘pleasant’ than patients without severe fatigue. Moreover, in patients with sarcoidosis fatigue severity, dyspnea, catastrophizing and depression were significantly associated with all four categories of the Fatigue Quality List that describe the experienced fatigue (P<0.05).ConclusionsThe current findings of experiences of fatigue in patients with IPF or pulmonary sarcoidosis provide insights for professionals treating these patients. Although similarities were found in the several experiences of fatigue across non-severely and severely fatigued patients, differences were also evident and could be mapped for IPF and sarcoidosis.     

Several specific high-resolution computed tomography patterns correlate with survival in patients with idiopathic pulmonary fibrosis

BackgroundEvidence of honeycombing in high-resolution computed tomography (HRCT) is a recognized risk factor for shortened survival in patients with idiopathic pulmonary fibrosis (IPF), but few studies have evaluated the feasibility of exploiting other specific patterns for predicting survival. The aim of this study was to examine the extent of specific HRCT patterns in IPF and determine whether they correlate with clinical features, pulmonary function tests (PFT), and survival.MethodsBoth the presence and extent of specific HRCT patterns, such as traction bronchiectasis, honeycombing, architectural distortion, reticulation, emphysema, and ground glass opacity, in 129 HRCT examinations were scored semi-quantitatively in three zones of each lung. HRCT examinations were also re-classified according to the 2011 and 2018 international statements. Correlations were calculated between the scores of specific HRCT patterns, clinical features, PFT, and patient survival.ResultsThe extent of traction bronchiectasis was found to be an independent risk factor of shortened survival (HR 1.227, P=0.001). Patients with a possible usual interstitial pneumonia (UIP) pattern had a better median survival than the patients with a definite UIP pattern (61 vs. 37 months, P=0.026). The extents of traction bronchiectasis, honeycombing, and architectural distortion displayed an inverse correlation with all PFT values at the time of diagnosis. There were few differences between the radiological classifications of the 2011 and 2018 international statements.ConclusionsWe conclude that several specific HRCT patterns displayed a correlation with shortened survival in IPF; these may help in evaluating the risk of death in IPF patients.     

肺结核患者外周血单个核细胞中miRNA-150的表达及意义

目的分析外周血单个核细胞(PBMC)中miRNA-150在肺结核患者(TB)及健康人群(HD)中的表达差异,探讨miRNA-150诊断活动性肺结核的应用价值。方法选择41例TB患者为实验组(其中19例为涂阳患者,22例为凃阴患者),同时选择35例HD为对照组。采集外周抗凝血利用密度梯度离心法分离PBMC,提取总RNA,利用Taq Man探针q PCR技术检测miRNA-150在各组人群中的相对表达量,以RNU6B作为内参基因。结果 miRNA-150在TB患者外周血PBMC中表达量为(8.74±1.36),显著高于健康对照组(3.544±0.59),差异有统计学意义(P0.05);应用ROC曲线分析表明,miRNA-150诊断TB的敏感性为61%,特异性为82%。痰涂片阳性TB患者中miRNA-150表达水平显著高于痰涂片阴性患者(12.30±2.43Vs 5.66±1.12),差异有统计学意义(P0.05)。结论 miRNA-150在TB患者PBMC中表达水平升高,尤其是涂阳患者,可以作为诊断TB的分子标识。     

Diagnostic usefulness of bronchoscopy for peripheral pulmonary lesions in patients with idiopathic pulmonary fibrosis

BackgroundAs lung cancers arising in a background of idiopathic pulmonary fibrosis (IPF) are known to show high malignancy grades, early pathologic diagnosis of peripheral pulmonary lesions (PPLs) is important. Meanwhile, the risk of complications associated with diagnostic procedures is high, which prompted us to investigate the role of bronchoscopy, a relatively safe diagnostic procedure. Therefore, we conducted this study to evaluate the usefulness of bronchoscopy for the diagnosis of PPLs in patients with IPF.MethodsData of consecutive patients with IPF who underwent bronchoscopy under radial endobronchial ultrasound (R-EBUS) guidance for PPLs at our institution between April 2014 and March 2019 were retrospectively reviewed. IPF was defined as usual interstitial pneumonia (UIP) or probable UIP, in accordance with the classification in the latest global guidelines. The diagnostic outcomes and the factors independently related to the diagnostic yield were analyzed.ResultsA total of 92 patients were included in the analysis. The median (range) size of the target PPLs was 27.1 (11.4–75.3) mm, and the diagnostic yield was 82.6%. Multivariable analysis identified a larger size [P=0.017; odds ratio (OR), 5.33; 95% confidence interval (CI), 1.29–22.01], positive bronchus sign (P=0.035; OR, 4.99; 95% CI, 1.12–22.18), and not involved with UIP/probable UIP pattern (P=0.023; OR and 95% CI, unmeasurable) as being associated with a significantly higher diagnostic yield. Meanwhile, none of the patients developed acute exacerbation of IPF or pneumothorax following the diagnostic bronchoscopy.ConclusionsBronchoscopy using R-EBUS was safe and showed an acceptable diagnostic yield for PPLs, even in patients with IPF.     

特发性肺纤维化患者外周血细胞产生IL-8能力的研究

目的　探讨肺纤维化与IL 8的关系。方法　采用ELISA法测定 2 4例IPF患者及 15例健康人PMBCs产生IL 8的能力。结果　IPF患者PBMCs产生IL 8为 (2 6 5 6 6± 35 32 )ng/L ,明显高于健康对照组 (180 0 9±32 4 6 )ng/L(P <0 0 1)。结论　IPF的发病与IL 8密切相关。     

肺活检在特发性肺纤维化诊断中的应用

特发性肺纤维化(IPF)是特发性间质性肺疾病中最常见、预后最差的类型.早诊早治是改善患者预后的关键措施.对于胸部高分辨率CT(HRCT)表现不是典型的普通型间质性肺炎(UIP)时,通常需要进行肺活检辅助诊断.外科肺活检可获取到足够的样本,是获得肺组织学样本的金标准.但外科肺活检可能导致IPF患者病情急性加重,甚至死亡....     

乙型肝炎患者外周血单个核细胞端粒酶活性的研究

目的 了解乙型肝炎患者外周血单个核细胞（PBMC）端粒酶活性的表达情况。方法 通过扩增端粒重复序列（TRAP）及光度酶联免疫法，分别检测健康人及各类乙型肝炎患者PBMC的端粒酶水平。结果 各组患者PBMC在植物血凝素（PHA）刺激前均有端粒酶活性的表达，以急性型肝炎组最高，重型肝炎组最低，二者差别具有显著性（P〈0．001）。PHA刺激后与刺激前比较各组端粒酶活性均有显著性升高（P〈0．001），刺激后的端粒酶水平以重型肝炎组为最低，与其他三组比较差别具有显著性（P〈0．05）。慢性重型乙型肝炎经胸腺五肽（TP5）治疗后端粒酶活性显著增高（P〈0．05）。结论 HBV急性感染期PBMC的端粒酶水平升高；慢性感染期PBMC的端粒酶水平在体内被抑制。TP5具有免疫调节作用，能使过低的端粒酶水平趋向于正常。     

肝细胞癌患者外周血单个核细胞Foxp3 mRNA水平变化*

目的 检测初发现未治疗肝细胞癌（HCC）患者外周血单个核细胞（PBMCs）中 Foxp3 mRNA水平变化及其与血清AFP水平的关系。方法 在HCC患者60例和健康人20例,采用RT-PCR法检测PBMCs中Foxp3 mRNA水平,常规检测血清AFP水平。结果 HCC患者和健康对照组外周血Foxp3 mRNA水平分别为（0.976±0.073）和（0.772±0.083）,差异有统计学意义（P<0.01）;经Pearson相关性分析显示,HCC患者外周血Foxp3 mRNA水平与AFP水平呈正相关（r＝0.226,P＝0.025）。结论 HCC患者PBMCs 中Foxp3水平明显升高,对肿瘤的发生发展可能具有一定的预测作用。