大剂量N-乙酰半胱氨酸治疗特发性肺间质纤维化的临床效果分析

目的观察大剂量N-乙酰半胱氨酸治疗特发性肺间质纤维化的临床效果。方法选取时间2014年4月20日—2017年4月20日四川省雅安市人民医院收取的80例特发性肺间质纤维化患者,采取随机的方式将本院所有观察对象分为两组,各40例。对照组采用常规治疗,观察组采用大剂量N-乙酰半胱氨酸治疗,比较两组治疗效果、临床指标改善情况。结果观察组特发性肺间质纤维化患者治疗总有效率为97.50%,与对照组比较,差异具有统计学意义(P0.05)。观察组特发性肺间质纤维化患者临床指标改善情况均优于对照组(P0.05)。结论采用大剂量N-乙酰半胱氨酸治疗特发性肺间质纤维化患者取得了显著的疗效,同时还能有效改善患者各项临床指标,值得研究。     

特发性肺间质纤维化发病机制及治疗进展

特发性肺间质纤维化（idiopathic pulmonary fibrosis,IPF）是特发性间质性肺炎（idiopathic interstitial pneumonia,IIP）中最常见的一种,由于其发病机制复杂,目前尚无有效治疗措施,预后极差,成为目前国内外的诊治难点及研究热点。近年来,在IPF发病机制及药物治疗方面的认识取得了一些进展。     

环磷酰胺、大剂量N-乙酰半胱氨酸治疗特发性肺间质纤维化疗效分析

目的探讨特发性肺间质纤维化的临床有效治疗方法。方法将65例特发性肺间质纤维化患者分为治疗组1、治疗组2和对照组三组。对照组给予常规强的松治疗;治疗组1给予强的松加环磷酰胺治疗;治疗组2给予强的松加大剂量N-乙酰半胱氨酸治疗。观察并比较患者治疗前后临床症状体征、胸部影像学及肺功能的变化。结果治疗前各组间具有可比性。治疗后,治疗组1与对照组的疗效无明显差异;而治疗组2的疗效则明显好于治疗组1及对照组。结论强的松联合大剂量N-乙酰半胱氨酸是临床治疗特发性肺间质纤维化的有效手段。     

PM2.5与特发性肺间质纤维化相关性的研究进展

<正>特发性肺间质纤维化(Idiopathic Pulmonary Fibrosis,IPF)是一种不可逆的肺组织结构损伤性疾病,预后差,生存期短,故该疾病防重于治。近年来多项动物实验及临床研究也证实PM2.5暴露与肺间质纤维化的发病及进展有关,故本文着重就PM2.5与特发性肺间质纤维化的相关性作如下综述。特发性肺间质纤维化特发性肺间质纤维化是间质性肺炎中较为常见的类型,病因尚不完全明确,好发于60岁以上的老     

85例间质性肺疾病临床特征分析

目的探讨间质性肺疾病临床特点诊断及治疗。方法回顾性分析85例肺间质纤维化患者的临床特点和肺功能及治疗情况。结果两组患者肺功能改变主要为限制性的通气障碍。49例特发性肺间质纤维化（IPF）者,具有进行性呼吸困难和爆裂音杵状指,CT／HRCT表现比较典型。36例继发性肺间质纤维化者伴有原发病症状。结论特发性肺间质纤维化和继发性肺间质纤维化的临床特征各不同,及时诊断对指导治疗及判断疾病进展和预后具有重要意义。     

甲基强的松龙联合阿奇霉素治疗特发性肺间质纤维化的临床疗效

特发性肺间质纤维化是一种以弥漫性肺泡炎和肺泡结构紊乱,最终导致肺间质纤维化,可发生呼吸衰竭的疾病。发病机制未完全清楚。目前尚无特效药物治疗。1999年10月~2004年10月,我们采用甲基强的松龙联合阿奇霉素治疗特发性肺间质纤维化36例,疗效尚可,报告如下。1资料与方法36例住院患者,男21例,女15例,年龄40~78岁,平均57.3岁。发病时间多在半月至7个月不等。诊断均符合中华医学会“特发性肺间质纤维化诊断与治疗”试行方案[1]。本组36例患者均经纤维支气管镜肺活检或经皮肺活检病理证实。给予甲基强的松龙0.5m g(kg·d),用药3~4周,阿奇霉素50…     

老年特发性肺间质纤维化21例影像学表现

目的 分析老年特发性肺间质纤维化影像学特点,探讨影像学资料在临床诊断中的意义.方法 对2005年1月至2011年3月我院收治的21例老年特发性肺间质纤维化老年患者的诊治资料进行回顾性分析,总结老年特发性肺间质纤维化患者的影像学表现特点.结果 21例患者均经病理活检证实为特发性肺间质纤维化.高分辨率CT表现为多样性,7例表现为肺气肿样;3例表现为蜂窝状影;9例表现为网格状影;2例表现为磨玻璃样影.胸片显示两肺为蜂窝状影和网格状影.结论 老年特发性肺纤维化高分辨率CT影像表现比较典型,老年病人合并有慢性阻塞性肺疾病时,需注意和慢性支气管炎肺纤维化区分开.     

如何早期确诊间质性肺疾病疑似病例的临床诊断思路与程序

<正>间质性肺疾病(interstitial lung disease,ILD)又称为弥慢性实质性肺疾病(diffuse parenchymal lungdisease,DPLD)是一大类疾病,包括200多个病种,肺间质纤维化是ILD一个结局性或终末期病理改变,此期激素治疗效果差,而目前尚无有效逆转纤维化的药物。并且,多数ILD起病隐匿,早期不易发现,一经确诊,纤维化已形成,往往表现为右心衰竭、呼吸衰竭,严重威胁生命。文献报道英格兰每年新增特发性肺间质     

全国慢性肺间质性疾病专题研讨会暨全国慢性肺间质性疾病学习班征文通知

由中华医学会呼吸病学分会主办的“全国慢性肺间质性疾病专题研讨会暨全国慢性肺间质性疾病学习班” ,拟于 2 0 0 1年10月在福州召开。届时将邀请著名专家作有关慢性肺间质性疾病的基础和临床研究的专题报告 ,现将会议征文的内容和要求通知如下。征文内容 :(1)慢性肺间质病的临床分类 ;(2 )特发性肺间质纤维化与寻常性间质性肺炎的发病学 ;(3)特发性肺间质纤维化的诊断 (ＢＡＬ ,ＴＢＬＢ ,影像学 ,肺功能 ) ;(4 )特发性肺间质纤维化的治疗 ;(5 )特发性肺间质纤维化的病理学 ;(6 )非特异性间质性肺炎的临床与治疗 ;(7)结节病的诊断与治疗 ;(…     

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.     

Pulmonary hypertension in idiopathic pulmonary fibrosis: a review

Idiopathic pulmonary fibrosis (IPF) is a progressive diffuse parenchymal disease with a poor prognosis. Pulmonary hypertension (PH) often complicates the course of IPF and may even be found in patients with preserved lung function. Possible pathogenetic mechanisms of PH in IPF include vascular destruction, pulmonary hypoxic vasoconstriction and vascular remodeling due to overexpression of cytokines and growth factors. PH in IPF patients is associated with decreased exercise capacity and a worse prognosis. Due to its prognostic significance, it seems important to investigate for PH in these patients. As the symptoms of PH in IPF are nonspecific, the development of PH in a patient with known IPF can be easily overlooked. Noninvasive methods provide clues for the diagnosis, but their sensitivity is limited. Doppler echocardiography is a useful tool for the detection of PH which also provides additional information regarding associated cardiac abnormalities. However, right heart catheterization remains the gold standard diagnostic test. Therapeutic options for PH in IPF are limited. Long-term oxygen administration for the correction of hypoxemia should be recommended. The availability of new pharmacological agents in the treatment of PH has raised the possibility of therapy in patients with IPF and associated PH. Whether these PH-targeted therapies may be of benefit in this patient group, in terms of improving functional outcomes and survival, remains uncertain.     

Idiopathic pulmonary fibrosis: phenotypes and comorbidities

Idiopathic pulmonary fibrosis (IPF) is a progressive fatal disease of the lung with an unknown etiology and limited treatment options. Three distinct phenotypes of IPF have been proposed: combined pulmonary fibrosis and emphysema, disproportionate pulmonary hypertension in IPF, and rapidly progressive IPF. Although treatment options for IPF are limited, much can be done to identify and alleviate symptoms from comorbidities, potentially improving the overall quality of life and well-being of these patients. This article describes emerging evidence to support the hypothesis that there is more than one phenotype for IPF and describes the common comorbidities seen in this disease.     

Pirfenidone: significant treatment effects in idiopathic pulmonary fibrosis

Pirfenidone has been shown in three recently published trials to slow down the progression of the devastating interstitial lung disease, idiopathic pulmonary fibrosis (IPF). The precise mechanisms that initiate and perpetuate the histopathological process leading to lung fibrosis in IPF are still uncertain, but increased concentrations of reactive oxidative species and fibrogenetic factors have been observed in the pulmonary tissue of patients. Although the exact mechanisms of its action are unknown, pirfenidone is a small molecule with antifibrotic and some hydroxyl scavenger properties that has recently been approved in Europe and elsewhere for the treatment of IPF. Along with the new ATS/ERS/JRS/ALAT 2011 statement for 'Evidence Based Guidelines for Diagnosis and Management', there is now a more profound basis for offering IPF patients an evidence-based evaluation and treatment. This review summarizes the background to the recommended use of pirfenidone for the treatment of IPF.     

Molecular targets in pulmonary fibrosis: the myofibroblast in focus

Idiopathic pulmonary fibrosis (IPF) is one of a group of interstitial lung diseases that are characterized by excessive matrix deposition and destruction of the normal lung architecture. Long-term survival of IPF patients is poor, with a 5-year survival rate of only 20%. Despite a lack of evidence-based benefit, IPF has historically been treated with corticosteroids and/or cytotoxic agents such as prednisone. Given the poor efficacy of these drugs, novel therapeutic strategies are required for the management of IPF. This demands a better understanding of the molecular mechanisms underlying the pathogenesis and progression of this disease. The primary effector cell in fibrosis is the myofibroblast; these cells are highly synthetic for collagen, have a contractile phenotype, and are characterized by the presence of alpha-smooth muscle actin stress fibers. They may be derived by activation/proliferation of resident lung fibroblasts, epithelial-mesenchymal differentiation, or recruitment of circulating fibroblastic stem cells (fibrocytes). From a therapeutic viewpoint, interfering with the pathways that lead to myofibroblast expansion should be of considerable benefit in the treatment of IPF. This review will highlight some of the key molecules involved in this process and the clinical trials that have ensued.     

Expectation of new treatments for idiopathic interstitial pneumonias

Idiopathic pulmonary fibrosis (IPF), a chronic form of idiopathic interstitial pneumonia, has a poor prognosis with an average life expectancy of 3-4 years from the time of diagnosis. Although patients with IPF have been treated with steroids or immunosuppressants to control the inflammation that occurs earlier in the course of disease, these drugs have not improved the survival of patients with IPF. Recently, several clinical studies of antifibrotic drugs have been conducted in patients with IPF. In Japan, we demonstrated that pirfenidone prevents deterioration of pulmonary function and significantly decreases the incidence of acute exacerbation of IPF in a well-designed, placebo-controlled, double-blind, randomized study. We have also adopted a new system for evaluation of dynamic pulmonary function, which involves measuring the lowest SpO2 level during a 6-minute walk at a constant speed and assessing the pulmonary capacity of patients with IPF. In a study of patients with pulmonary fibrosis associated with Hermansky-Pudlak syndrome in the United States, pirfenidone slowed the decrease in %FVC in patients with a %FVC of >60%, but had no significant effects on patients with a %FVC of < or =60%. Large-scale clinical studies of INF-gamma in patients with IPF in North America and Europe have reported decreases in the mortality of patients with mild IPF with a FVC of >60%, although percentages of patients with disease status rated as 'exacerbated', 'unchanged' and 'improved' after treatment did not differ between the INF-gamma and placebo groups. This presentation reported important future strategies for the treatment of IPF.     

Abnormal lung aging in chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis

Aging is a natural process characterized by progressive functional impairment and reduced capacity to respond appropriately to environmental stimuli and injury. The incidence of two common chronic respiratory diseases (chronic obstructive pulmonary disease [COPD] and idiopathic pulmonary fibrosis [IPF]) increases with advanced age. It is plausible, therefore, that abnormal regulation of the mechanisms of normal aging may contribute to the pathobiology of both COPD and IPF. This review discusses the available evidence supporting a number of aging mechanisms, including oxidative stress, telomere length regulation, cellular and immunosenescence, as well as changes in a number of antiaging molecules and the extracellular matrix, which are abnormal in COPD and/or IPF. A better understanding of these abnormalities may help in the design of novel and better therapeutic interventions for these patients.     

细胞因子网络与特发性肺纤维化

特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)是特发性间质性肺炎中最常见的一种类型,也是一种进展性的不可逆的快速致死性问质性肺疾病.关于IPF发病机制及治疗的研究已经有50余年,目前还没有有效的治疗方案能逆转肺纤维化.IPF的发病机制复杂,细胞因子网络是其重要机制.现已知生长因子、细胞因子、化学因子以及凋亡调节因子在肺纤维化发病机制中起重要作用.目前认为转化生长因子β是细胞因子网络的关键环节,与之相关的各种细胞因子是纤维化进程中的重要介质.本综述总结了IPF发病机制相关的细胞因子及其交互作用,通过对这些与异常损伤修复相关介质的阐述,为IPF靶向治疗提供理论依据.     

Do all patients with idiopathic pulmonary fibrosis warrant a trial of therapeutic intervention? A pro‐con perspective

Idiopathic pulmonary fibrosis (IPF) is an incurable condition that is characterized by progressive pulmonary fibrosis, architectural distortion of the lung and loss of gas exchange units. Until recently, there was no effective treatment for this condition. However, there were two landmark trials published earlier this year, which have changed the management of this condition. Pirfenidone (Assessment of Pirfenidone to Confirm Efficacy and Safety in Idiopathic Pulmonary Fibrosis trial) and nintedanib (Efficacy and Safety of Nintedanib in Idiopathic Pulmonary Fibrosis‐1 and ‐2 trials) have both demonstrated positive outcomes in patients with IPF. In this perspective, we critically discuss the role of these agents in IPF and in the broader pulmonary fibrosis population.     

Idiopathic pulmonary fibrosis: a disorder of epithelial cell dysfunction

Idiopathic pulmonary fibrosis (IPF) is characterized by progressive dyspnea, interstitial infiltrates in lung parenchyma and restriction on pulmonary function testing. IPF is the most common and severe of the idiopathic interstitial pneumonias, with most individuals progressing to respiratory failure. Multiple lines of evidence reveal prominent roles for alveolar epithelial cells (AECs) in disease. The current disease paradigm is that ongoing or repetitive injurious stimuli in the presence of a genetic or acquired dysfunctional type II AEC phenotype results in increased AEC injury/apoptosis, deficiencies in regeneration of normal alveolar structure and aberrant lung repair and fibroblast activation, leading to progressive fibrosis. Although the nature of injurious events and processes involved in aberrant repair of the alveolar epithelium are not well understood, ongoing investigations provide hope to better understand mechanisms by which AECs maintain homeostasis or contribute to fibrosis. These strategies may hold promise for developing novel treatment approaches for IPF.     

Idiopathic pulmonary fibrosis: pathobiology of novel approaches to treatment

Idiopathic pulmonary fibrosis (IPF) is a progressive disease of unknown cause that conveys a dismal prognosis. In the United States there are currently no licensed therapies for treatment of IPF. The development of effective IPF clinical trials networks across the United States and Europe, however, has led to key developments in the treatment of IPF. Advances in understanding of the pathogenetic processes involved in the development of pulmonary fibrosis have led to novel therapeutic targets. These developments offer hope that there may, in the near future, be therapeutic options available for treatment of this devastating disease.