IL-8、CXCL14、IGFBP-2联合在特发性肺纤维化中的诊断价值

目的探讨白细胞介素(IL-8)、C-X-C Motif趋化因子配体14(CXCL14)、胰岛素样生长因子结合蛋白2(IGFBP-2)联合在特发性肺纤维化中的诊断价值。方法选取特发性肺纤维化患者30例为纤维组,细菌性肺炎患者30例为肺炎组,同时选取体检健康者30例为对照组,检测3组血清中IL-8、CXCL14、IGFBP-2的表达量。用受试者工作特征曲线(ROC曲线)下面积分析IL-8、CXCL14、IGFBP-2对特发性肺纤维化的诊断价值。结果特发性肺纤维化患者血清中IL-8、CXCL14和IGFBP-2的表达量均明显高于细菌性肺炎患者和体检健康者,差异有统计学意义(P0.05)。诊断特发性肺纤维化患者与细菌性肺炎患者IL-8、CXCL14、IGFBP-2的灵敏度分别为80%、77%、70%,特异度为80%、87%、87%;诊断特发性肺纤维化患者与体检健康人群IL-8、CXCL14、IGFBP-2的灵敏度分别为83%、97%、83%,特异度为83%、87%、87%;IL-8、CXCL14、IGFBP-2联合对特发性肺纤维化患者诊断的灵敏度为86.67%,特异度为83.33%,准确度为84.44%。结论 IL-8、CXCL14、IGFBP-2可作为联合诊断特发性肺纤维化的潜在标志物。     

特发性肺纤维化患者血清基质金属蛋白酶-9水平及其临床意义

目的探讨血清基质金属蛋白酶9水平对特发性肺纤维化患者早期诊断的价值。方法采用酶联免疫吸附法对32例特发性肺纤维化患者(早期组19例,非早期组13例)和24例健康人(健康对照组)血清基质金属蛋白酶9进行测定。结果早期组和非早期组血清基质金属蛋白酶9均高于健康对照组,差异有显著性意义(P<0.01);早期组血清基质金属蛋白酶9高于非早期组,两组间比较差异有显著性意义(P<0.01)。结论基质金属蛋白酶9在特发性肺纤维化发病机制中起着重要作用,血清基质金属蛋白酶9测定对特发性肺纤维化早期诊断具有一定价值。     

超声在肺纤维化中的应用进展

肺纤维化是一类肺间质疾病,可分为原因不明的特发性肺纤维化和继发于其他原因的肺纤维化.小部分患者病情可长期稳定,大部分患者病情进展缓慢,个别患者病情进展迅速.因此早期诊断和及时干预对改善肺纤维化患者预后至关重要.随着超声技术的发展,其在肺部疾病诊断、疗效评估及病情进展监测中的应用越来越多.本文就超声在肺纤维化诊断、治疗随...     

细胞因子治疗特发性肺纤维化

目的:总结并分析细胞因子在特发性肺纤维化发病机制与治疗中的作用,特别是致炎细胞因子在特发性肺纤维化治疗中的应用。资料来源:应用计算机检索Pubmed1996-01/2006-05有关细胞因子治疗特发性肺纤维化应用及研究的文献,检索词“idiopathic pulmonary fibrosis,cytokines,biologic agent therapy,gene therapy”,并限定文献语言种类为English。资料选择:对资料进行整理,并查看每篇文献后的引文,选取针对性强的文章,同一领域的文献则选择近期发表或权威杂志的文章。纳入标准:关于特发性肺纤维化的概念、分类、发病机制和治疗方法及细胞因子在特发性肺纤维化发病机制和治疗方法中的作用。排除标准:重复性研究。资料提炼:共检索到54篇符合要求的相关文献,其中研究内容相似的,以近3年内发表的文章优先。对符合标准的30篇文献进行分析。纳入的文章主要涉及细胞因子在特发性肺纤维化的发病机制和治疗方法中的作用。资料综合:目前,特发性肺纤维化的传统药物疗法仍然存在争议。随着对特发性肺纤维化发病机制认识的逐渐深入,各种新的治疗策略相继出现。而最近的临床和动物试验研究均提出针对调节促纤维化细胞因子的治疗是比较有前途的治疗策略。促纤维化细胞因子的释放导致纤维细胞增殖并迁移到肺组织的不同部位,接着分化为不同的成纤维细胞亚型,而这种纤维细胞的分化可能是特发性肺纤维化慢性进程的关键原因。针对细胞因子治疗包括生物制剂治疗和基因治疗两大领域已经成为特发性肺纤维化治疗研究的热点和主要方向。本文从细胞因子在特发性肺纤维化治疗中的应用方面进行综述。结论:细胞因子在特发性肺纤维化发病中起到了促纤维化的关键性作用,针对细胞因子包括生物制剂治疗和基因治疗两大领域已经成为治疗特发性肺纤维化最有前景的手段之一。     

特发性肺纤维化7例报告

特发性肺纤维化７例报告广州市第一人民医院（５１０１８０）刘朝晖钟维农曾军黄玉宇中山医科大学病理学教研室李扬特发性肺纤维化（ＩＰＦ）临床表现复杂，预后不良，发病率有逐年上升趋势，我院１９８８年～１９９４年经纤支镜肺活检、经皮肺活检及支气管肺泡灌洗共诊断...     

特发性肺纤维化的治疗进展

特发性肺纤维化( idiopathic pulmonary fibrosis，IPF)是一种以普通型间质性肺炎为特征性病理改变的慢性、弥漫性肺间质病变的疾病。其临床主要表现为进行性呼吸困难和刺激性干咳。特发性肺纤维化的研究进展缓慢，预后很差，诊断后的中位生存时间为4年，目前还没有有效的治疗手段。本文就其治疗研究的新进展做一综述。     

特发性肺纤维化患者的Th1／Th2细胞因子失衡

本研究检测特发性肺纤维化(IPF)患者血清IFN- γ和IL- 4水平,探讨特发性肺纤维化发病、治疗和预后与Th1/ Th2类细胞因子失衡的关系。1　对象和方法1.1　对象　2 0 0 2 - 0 9～2 0 0 3- 10在本院就医确诊特发性肺纤维化4 0例,其中男2 9例,女11例,年龄(4 5±9)岁,所有患者均接受口服强的松和环磷酰胺治疗3个月,其中16例治疗反应良好,2 4例失败,诊断标准、治疗方案及疗效判定见《特发性肺(间质)纤维化诊断和治疗指南(草案)》[1 ] ,不伴感染性或免疫失调性疾病。对照组为性别、年龄相当的健康体检者32例,其中男18例,女14例,年龄(4 0±7)岁。…     

肺纤维化患者血清及诱导痰肺表面活性蛋白水平测定及意义

目的检测特发性肺纤维化患者急性加重期血清及诱导痰肺表面活性蛋白D(SP-D)水平并探讨其临床治疗意义。方法选择特发性肺纤维化患者急性加重期住院治疗26例,分别收集纳入患者入院时及治疗后病情缓解血清及诱导痰液标本,采用酶联免疫吸附试验检测血清及诱导痰SP-D水平。选择42例健康人群作为对照组。结果特发性肺纤维化患者急性加重期及病情缓解期血清SP-D平均水平分别为(196±82)、(125±69)ng/mL,健康对照组为(96±47)ng/mL,诱导痰SP-D平均水平为(153±71)、(106±64)ng/mL,急性加重期与缓解期及健康对照组SP-D水平比较差异有统计学意义(P0.05)。结论血清SP-D检测可作为特发性肺纤维化患者急性加重诊断的生物学指标。     

CT测定肺动脉和主动脉比值作为特发性肺纤维化患者肺动脉高压诊断和预后指标的研究

目的:探讨CT测定肺动脉直径和主动脉直径比值(rPA)作为特发性肺纤维化患者肺动脉高压诊断和预后指标的效果。方法:选取2019年1月至2020年1月我院收治的特发性肺纤维化患者52例作为研究组,选取同期健康体检者45例作为对照组。两组入院时、出院时均检测肺动脉直径(dPA)与主动脉直径,计算dPA与主动脉直径比值(rPA),同时检测肺动脉压力(SPAP),并通过Pearson分析研究组患者入院时与出院时的rPA与同期SPAP的关系。结果:研究组患者入院时PASP、rPA均明显高于对照组健康者(P<0.05),出院时各指标均有明显下降,与对照组无明显差异(P>0.05);患者入院时与出院时的rPA与同期PASP呈正相关(P<0.05)。结论:肺动脉和主动脉比值与特发性肺纤维化患者的肺动脉压力具有相关性,在诊断特发性肺纤维化患者肺动脉高压中具有一定价值,也可作为预后的重要预测指标。     

特发性肺纤维化患者血清基质金属蛋白酶-9水平及其与肺功能相关意义

目的探讨血清基质金属蛋白酶-9水平和特发性肺纤维化患者肺功能的相关性。方法采用酶联免疫吸附法对40例特发性肺纤维化患者和30例健康人血清基质金属蛋白酶-9进行测定。结果患者血清基质金属蛋白酶-9水平高于健康对照组,差异有显著性意义（P〈0.01）;肺功能重度异常组血清基质金属蛋白酶-9高于轻、中度异常组,两组间比较差异有显著性意义（P〈0.01）。结论基质金属蛋白酶-9在特发性肺纤维化发病机制中起着重要作用,血清基质金属蛋白酶-9测定对判断特发性肺纤维化的严重程度具有一定价值。     

Treatments in idiopathic pulmonary fibrosis: time for a more targeted approach?

Idiopathic pulmonary fibrosis (IPF) is a progressive age-related lung disease, the cause of which is not been fully understood. IPF is a devastating disease with mortality worse than many cancers, and treatment options are limited. IPF is thought to occur after recurrent injury to the alveolar epithelium followed by abnormal repair characterized by the formation of fibroblast and myofibroblast foci and excessive deposition of extracellular matrix. An updated classification of the idiopathic interstitial pneumonias has encouraged a large number of clinical trials. On the whole, these have disappointed. Improvements in molecular techniques have developed our understanding of IPF and with it identified new pathways and potential targets for therapeutic intervention. These insights are leading to interest in biomarkers of disease progression and prognosis and to novel anti-fibrotic agents and a more targeted approach to the treatment of IPF.     

特发性肺纤维化合并肺动脉高压的诊治进展

特发性肺纤维化(idiopathic pulmonary fibrosis, IPF)是病因不明,组织病理学主要为普通间质性肺炎,局限于肺部的慢性纤维化型间质性肺炎。该病致死率高,对人群健康危害性高。近年来,随着人口老龄化、空气污染的加剧导致其发病率逐年上升,我们对于IPF的认识也在不断提高,研究发现IPF患者常合并其他疾病,包括肺气肿、肺癌、心血管疾病等。存在合并症的IPF患者临床症状更多,患者的生存质量差。其中合并肺动脉高压会降低IPF患者的生存率,本文就IPF合并肺动脉高压进行综述,以期为IPF合并肺动脉高压的诊疗方案与进一步研究提供新思路,以期提高 IPF患者的生活质量及改善预后。     

Interferon-gamma 1b for the treatment of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) represents a particularly aggressive disease, the aetiology of which still remains unknown. The natural history of the disease often leads to respiratory failure and death, with a mortality rate greater than some cancers. To date, no management approach has proven to be efficacious for the treatment of this disease. IPF has been characterised as an 'epithelial-fibroblastic disorder', characterised by abnormal wound healing with excessive fibrosis and minimal inflammation. These emerging data have focused attention on antifibrotic drugs. Interferon-gamma1b (IFN-gamma1b) has recently been proposed as a promising candidate for the treatment of IPF. The reason for this is that IFN-gamma1b has the ability to modulate the Th1/Th2 imbalance and to suppress fibroblast activation. The view that IPF is untreatable at present requires reconsideration, as improved survival has been suggested in three controlled trials of IFN-gamma1b in IPF therapy.     

Idiopathic Pulmonary Fibrosis: A Review of Disease,Pharmacological, and Nonpharmacological Strategies With a Focus on Symptoms,Function, and Health-Related Quality of Life

Despite several advances in treatment, idiopathic pulmonary fibrosis (IPF) remains a progressive, symptomatic, and terminal disease in patients not suitable for lung transplantation. With disease progression, IPF often leads to a constellation of symptoms, including dyspnea, cough, anxiety, and depression. Palliative care is appropriate to support these patients. However, traditional curriculum in palliative care has often focused on supporting patients with malignant disease, and clinicians are not universally trained to manage patients with progressive nonmalignant diseases such as IPF. Current antifibrotic therapies aim to slow disease progression but are not able to reduce symptoms or improve daily function and health-related quality of life (HRQL). Palliative care in this patient group requires an understanding of the clinical characteristics of IPF, comorbidities, common medications used, and nonpharmacological strategies that can be undertaken to improve daily function and HRQL. This review focuses on IPF management strategies and their effects on symptoms, exercise tolerance, HRQL, and survival. Pharmacological interactions and considerations related to commonly used palliative care medications are also reviewed. This review highlights the needs of patients with IPF and caregivers, psychosocial function, patient-reported assessment tools, and topics related to advance care planning.     

Immune dysregulation as a driver of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) affects hundreds of thousands of people worldwide, reducing their quality of life and leading to death from respiratory failure within years of diagnosis. Treatment options remain limited, with only two FDA-approved drugs available in the United States, neither of which reverse the lung damage caused by the disease or prolong the life of individuals with IPF. The only cure for IPF is lung transplantation. In this review, we discuss recent major advances in our understanding of the role of the immune system in IPF that have revealed immune dysregulation as a critical driver of disease pathophysiology. We also highlight ways in which an improved understanding of the immune system’s role in IPF may enable the development of targeted immunomodulatory therapies that successfully halt or potentially even reverse lung fibrosis.     

Clinical review: Idiopathic pulmonary fibrosis acute exacerbations - unravelling Ariadne's thread

Idiopathic pulmonary fibrosis (IPF) is a dreadful, chronic, and irreversibly progressive fibrosing disease leading to death in all patients affected, and IPF acute exacerbations constitute the most devastating complication during its clinical course. IPF exacerbations are subacute/acute, clinically significant deteriorations of unidentifiable cause that usually transform the slow and more or less steady disease decline to the unexpected appearance of acute lung injury/acute respiratory distress syndrome (ALI/ARDS) ending in death. The histological picture is that of diffuse alveolar damage (DAD), which is the tissue counterpart of ARDS, upon usual interstitial pneumonia, which is the tissue equivalent of IPF. ALI/ARDS and acute interstitial pneumonia share with IPF exacerbations the tissue damage pattern of DAD. 'Treatment' with high-dose corticosteroids with or without an immunosuppressant proved ineffective and represents the coup de grace for these patients. Provision of excellent supportive care and the search for and treatment of the 'underlying cause' remain the only options. IPF exacerbations require rapid decisions about when and whether to initiate mechanical support. Admission to an intensive care unit (ICU) is a particular clinical and ethical challenge because of the extremely poor outcome. Transplantation in the ICU setting often presents insurmountable difficulties.     

不同来源的外泌体对特发性肺纤维化的免疫调节作用

特发性肺纤维化（IPF）是一种慢性进行性的间质性肺病,其发病机制目前尚无定论。成纤维细胞直接参与IPF过程的理论已被广泛接受,同时研究认为IPF的形成、发展与炎症、免疫息息相关,主要表现为免疫细胞与成纤维细胞间的相互作用。外泌体是细胞间进行生物信息通信的重要载体,不同细胞来源的外泌体携带着不同的信号因子,可作为免疫细胞与成纤维细胞的桥梁,在IPF的炎症反应和免疫调节中发挥作用,同时外泌体或将成为临床诊断和治疗IPF的重要方法。该文就不同细胞来源的外泌体在IPF中的研究进展进行综述,以了解外泌体作为细胞间信息交流的载体在调节IPF的形成与发展中的作用。     

影响特发性肺纤维化治疗和预后的常见合并症

特发性肺纤维化(idiopathic pulmonary fibrosis, IPF)是一种慢性进展性致纤维化性疾病, 预后极差。IPF以中老年男性多见, 容易出现多种临床合并症, 包括肺气肿、肺癌、心血管疾病等。存在合并症的IPF患者临床症状更多、预后更差。本文详细阐述影响特发性肺纤维化治疗和预后的常见合并症, 以期提高临床对于IPF合并症的认识和诊疗水平, 从而改善IPF患者的生活质量及预后。     

Interferon-γ1b for the treatment of idiopathic pulmonary fibrosis

Idiopathic pulmonary fibrosis (IPF) represents a particularly aggressive disease, the aetiology of which still remains unknown. The natural history of the disease often leads to respiratory failure and death, with a mortality rate greater than some cancers. To date, no management approach has proven to be efficacious for the treatment of this disease. IPF has been characterised as an ‘epithelial–fibroblastic disorder’, characterised by abnormal wound healing with excessive fibrosis and minimal inflammation. These emerging data have focused attention on antifibrotic drugs. Interferon-γ_1b (IFN-γ_1b) has recently been proposed as a promising candidate for the treatment of IPF. The reason for this is that IFN-γ_1b has the ability to modulate the Th1/Th2 imbalance and to suppress fibroblast activation. The view that IPF is untreatable at present requires reconsideration, as improved survival has been suggested in three controlled trials of IFN-γ_1b in IPF therapy.     

Plasma CCN2 (connective tissue growth factor; CTGF) is a potential biomarker in idiopathic pulmonary fibrosis (IPF)

BackgroundIdiopathic pulmonary fibrosis (IPF) is a chronic, progressive and fatal pulmonary fibrotic disease and useful biomarkers are required to diagnose and predict disease activity. CCN2 (connective tissue growth factor; CTGF) has been reported as one of the key profibrotic factors associated with transforming growth factor-β (TGF-β), and its assay has potential as a non-invasive measure in various fibrotic diseases. Recently, we developed a new subtraction method for determination of plasma CCN2 levels. We examined the utility of plasma CCN2 levels as a surrogate marker in IPF.MethodsPlasma CCN2 levels were calculated in 33 patients with IPF, 14 patients with non-IPF idiopathic interstitial pneumonias (IIPs) and 101 healthy volunteers by sandwich enzyme-linked immunosorbent assay (ELISA) using specific monoclonal antibodies for two distinct epitopes of human CCN2. We evaluated the utility of plasma CCN2 levels by comparison with clinical parameters.ResultsPlasma CCN2 levels were significantly higher in patients with IPF than in those with non-IPF IIPs and healthy volunteers. Importantly, plasma CCN2 levels showed significantly negative correlation with 6-month change of forced vital capacity (FVC) in patients with IPF.ConclusionsPlasma CCN2 is a potential biomarker for IPF.