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     

隐原性致纤维化肺泡炎的治疗

隐原性致纤维化肺泡炎(IFA)又称特发性弥漫性肺间质纤维化、特发性间质性肺炎和弥漫性间质性肺炎等。70年代以后国外已趋向用IFA这一病名,但尚未统一。笔者认为IFA既能说明本病的病理形成过程又能概括其临床特点,因而赞成使用IFA取代其它诊断名称。     

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 ;(…     

特发性肺纤维化诊断的研究进展

特发性间质性肺炎(ⅡP)是一组原因不明的肺间质性疾病,以进展性纤维化性炎症反应为特征.2002年ATS/ERS将IIP分为7大类,其中病理学表现为普通型问质性肺炎(UIP)的特发性肺纤维化(IPF)在IIP中所占比例最高、预后最差,且无特效治疗方法.     

弥漫性肺间质纤维化临床诊断思路

正弥漫性肺间质纤维化是一大类疾病,目前认为这类疾病至少具有以下四个特点:1病因繁多:据不完全统计其病因大约有180多种。已知病因包括职业性肺病(尘肺)、药物性肺病、结缔组织疾病相关性ILD(CTD-ILD)等。特发性间质性肺炎包括特发性肺纤维化(IPF)、非特异性间质性肺炎(NSIP)、隐源性机化性肺炎(COP)、急性间质性肺炎(AIP)、呼吸性细支气管炎间质性肺病(RB-ILD)、脱屑性间质性肺炎(DIP)和淋巴细胞性间质性肺炎(LIP)等。     

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。     

以肺部结缔组织病变为首发的间质性肺病1例及文献复习

特发性间质性肺炎（idiopathic interstitial pneumonia,IIP）是一组以肺间质和肺泡腔具有不同形式和程度的炎症和纤维化为主要病理特征的疾病。     

与特发性间质性肺炎相似的弥漫性实质性肺疾病的鉴别诊断

特发性间质性肺炎(idiopathic interstitial pneumonia,IIP)是一组以肺间质和肺泡腔具有不同形式和程度的炎症和纤维化为主要病理特征的疾病,其原因不明.     

特发性间质性肺炎的现代诊治

特发性间质性肺炎（Idiopathic interstitial pneumonias，IIPs）是一组发生在肺实质的不同形式和程度的炎症和纤维化所导致的异质性非特异性疾病，包括特发性肺间质纤维化（idiopathic pulmonary fibrosis，IPF）及其以外的其它间质性肺炎，发病原因不明，是间质性肺疾病（interstitial lung disease，ILD）或称弥漫性肺实质病变（diffuse parenchymal lung disease，DPLD）中最常见和最重要的疾病。     

特发性肺间质纤维化发病机制最新进展

特发性肺间质纤维化（idiopathic pulmonary fibrosis IPF）是弥漫性间质性肺病中的一种特殊类型,是一种原因不明、出现在成人、局限于肺、进行性致纤维化的间质性肺炎,其临床诊断主要依靠病理,美国ATS最新指南指出将HRCT列为独立诊断标准之一,     

Nonspecific interstitial pneumonia (NSIP)

Nonspecific interstitial pneumonia (NSIP) represents one histologic subtype of idiopathic interstitial pneumonia (IIP). NSIP is typified by temporal homogeneity and less profusion of fibroblastic foci than is seen with usual interstitial pneumonia (UIP), the most common IIP. Clinically patients with NSIP present with similar symptoms (cough and dyspnea) when compared to patients with UIP. The duration of these symptoms prior to presentation is variable. The finding of fever may be more common in NSIP and clubbing may be more common in UIP; however, both findings can be seen in either UIP or NSIP. Physiological findings typically demonstrate a restrictive ventilatory defect with decreased gas transfer; little difference exists between UIP and NSIP. High resolution computed tomography (HRCT) scans are more likely to show honeycombing with UIP and a ground-glass pattern with NSIP, however, either of these findings can be seen with UIP or NSIP. The most striking differential feature between NSIP and UIP is the markedly better prognosis for patients with NSIP, a finding that cannot be explained by baseline differences in physiology or radiographic features. In this article we explore the clinical, physiological, and radiographic features of NSIP. We also review available information regarding response to therapy and prognosis.     

Nonspecific interstitial pneumonia: a provisional category of idiopathic interstitial pneumonia

PURPOSE OF REVIEW: Idiopathic interstitial pneumonias (IIP) represent a complex group of relatively rare entities with similar clinical, vaguely similar radiographic and differing histologic features. The recent international multidisciplinary consensus statement produced by the American Thoracic Society/European Respiratory Society aiming to standardize the classification of IIP recognizes nonspecific interstitial pneumonia (NSIP) as a provisional category. While not representing a single disease, but rather a collection of pathologic processes with similar histomorphology, NSIP has been a great source of confusion for pulmonologists, radiologists, and pathologists. RECENT FINDINGS: Lacking diagnostic clinical or radiographic features, NSIP is an IIP with recognizable and reproducible morphologic patterns different from usual interstitial pneumonia (UIP)-pattern as well as other disease patterns. And while overlap with UIP-pattern can be seen in individuals with multiple biopsy samples, those with either cellular or fibrosing variants of NSIP have a better prognosis than UIP-pattern patients. SUMMARY: A morphologic diagnosis of NSIP-pattern alerts the clinician to a wide spectrum of potential clinical possibilities and enables researchers to study both this fibrosing interstitial pneumonia pattern and the more common and deadly UIP-pattern separately. Thus, this provisional category is useful to both clinicians and researchers.     

Nonspecific interstitial pneumonia: a real clinical entity?

Based on the current multidisciplinary classification of idiopathic interstitial pneumonia (IIP) organized by ATS/ERS, nonspecific interstitial pneumonia (NSIP) is considered as one type of IIP. An incidence of idiopathic NSIP is relatively small and possesses clinical features that are different than idiopathic pulmonary fibrosis (IPF) and usual interstitial pneumonia (UIP). Because there is little evidence of a long-term prognosis in patients with NSIP, some of them have an unfavorable prognosis similar to IPF/UIP. We review the significance of prognostic factors that have been reported in patients with IPF/UIP by applying them to patients with NSIP. The association with collagen vascular diseases focuses on etiologic background. Finally, the article discusses whether NSIP could be an early lesion of UIP based on the reported evidence and our own professional experiences.     

A comparative study of the prognosis for Japanese patients with idiopathic interstitial pneumonia or BOOP based on histopathologic subsets]

We explored the prognosis for 123 patients with either idiopathic interstitial pneumonia (IIP) or bronchiolitis obliterans organizing pneumonia (BOOP). All patients underwent either open lung biopsy or thoracoscopic lung biopsy procedures. The histopathologic diagnosis of IIP included patients with usual interstitial pneumonia (UIP), nonspecific interstitial pneumonia (NSIP), and desquamative interstitial pneumonia with respiratory bronchiolitis-associated interstitial lung disease. The prognosis was poorest for patients with a histologic diagnosis of UIP, and excellent for those who received a diagnosis of BOOP. Although the prognosis is generally considered to be good for patients with NSIP, some NSIP patients in our study died. Histopathologic diagnosis based on surgical lung biopsy is useful in evaluating the prognosis for patients with IIP.     

Prognosis of fibrotic interstitial pneumonia: idiopathic versus collagen vascular disease-related subtypes

BACKGROUND: To investigate whether the better prognosis of interstitial pneumonias associated with collagen vascular disease (CVD) compared with idiopathic interstitial pneumonia (IIP) is due to higher frequency of the nonspecific interstitial pneumonia (NSIP) pattern in CVD, we compared the outcomes of patients from these two groups with the same histopathologic pattern. SUBJECTS: The clinical features and survival of 362 patients (269 with IIP and 93 with CVD) diagnosed using surgical lung biopsy were analyzed. RESULTS: The mean survival of the CVD group (131.0 mo) was longer than that of the IIP group (80.5 mo) (p<0.0001). The patients with usual interstitial pneumonia pattern among the CVD group (n=36) was younger, female, and predominantly nonsmoking compared with the IIP group (n=203). Although baseline lung functions were not significantly different, the CVD group survived longer (mean, 177.0 mo) than the IIP group (mean, 66.9 +/- 6.5 mo; p=0.001). By multivariate analysis, younger age, better pulmonary function, and the presence of a CVD were independent prognostic factors. In NSIP pattern, no significant differences in survival, clinical features, or lung function were found between the two groups. CONCLUSION: Our data suggest that the better prognosis of patients in the CVD group is not solely due to the predominance of the NSIP pattern. The prognosis of patients with the usual interstitial pneumonia pattern in CVD is better than in those with idiopathic pulmonary fibrosis, despite the same pathologic pattern. In contrast, in those with an NSIP pattern, the prognosis is similar in both groups.     

Significance of connective tissue disease features in idiopathic interstitial pneumonia

In idiopathic interstitial pneumonia (IIP), the significance of connective tissue disease (CTD) features in the absence of a specific CTD diagnosis remains unclear. We studied the clinical and prognostic utility of a diagnosis of undifferentiated CTD (UCTD) in patients with biopsy-proven IIP. IIP patients undergoing surgical lung biopsy (1979-2005) were studied (nonspecific interstitial pneumonia (NSIP), n = 45; idiopathic pulmonary fibrosis, n = 56). UCTD was considered present when serum autoantibodies were present and symptoms or signs suggested CTD. The relationship between UCTD and NSIP histology was evaluated. A clinical algorithm that best predicted NSIP histology was constructed using a priori variables. The prognostic utility of UCTD, and of this algorithm, was evaluated. UCTD was present in 14 (31%) NSIP and seven (13%) IPF patients. UCTD was not associated with a survival benefit. The algorithm predictive of NSIP (OR 10.4, 95% CI 3.21-33.67; p<0.0001) consisted of the absence of typical high-resolution computed tomography (HRCT) features for IPF and 1) a compatible demographic profile (females aged <50 yrs) or 2) Raynaud's phenomenon. In patients with an HRCT scan not typical for IPF, this algorithm predicted improved survival (hazard ratio 0.35, 95% CI 0.14-0.85; p = 0.02) independent of IIP severity. UCTD is associated with NSIP histology. However, the diagnostic and prognostic significance of UCTD in IIP patients remains unclear.     

Characteristic elevation of matrix metalloproteinase activity in idiopathic interstitial pneumonias

Destruction of subepithelial basement membrane is a key event in the pathogenesis of idiopathic pulmonary fibrosis (IPF). To evaluate the role of matrix metalloproteinases (MMPs) in parenchymal remodeling in idiopathic interstitial pneumonia (IIP), we studied MMP-2 and -9 activity, in bronchoalveolar lavage fluid (BALF) by zymography and the expression of MMP-2 and -9 and TIMP-2 in lung tissue by immunohistochemistry. BALF and lung tissues were collected from 26 patients with usual interstitial pneumonia (IPF-UIP), 11 with nonspecific interstitial pneumonia (NSIP), and 6 with bronchiolitis obliterans organizing pneumonia (BOOP). IPF-UIP cases showed predominant expression of MMP-9, whereas NSIP and BOOP cases showed predominant MMP-2 expression in BALF and in tissues. In BALF samples from rapidly progressive IPF-UIP cases, neutrophil-derived MMP-9 activity, as well as MMP-9 active form were characteristically detected. Furthermore, the MMP-9 activity correlated significantly with an increase of neutrophils in BALF, whereas the MMP-2 activity associated with NSIP and BOOP correlated with an increase of lymphocytes. These results indicate that MMP-9 in IPF-UIP and MMP-2 in NSIP and BOOP may contribute to pulmonary structural remodeling through type IV collagenolytic activity. The characteristic contributions of matrix-degrading proteins may relate to the distinct prognostic features of these diseases.     

Classification and recent advances in idiopathic interstitial pneumonia

Idiopathic interstitial pneumonia (IIP) is a heterogeneous group of diseases comprising acute interstitial pneumonia, bronchiolitis obliterans organizing pneumonia (BOOP), nonspecific interstitial pneumonia, desquamative interstitial pneumonia, and idiopathic pulmonary fibrosis and usual interstitial pneumonia (IPF/UIP). We review the clinicopathological spectrum of IIP and introduce recent advances in classification, treatment, and prognosis. BOOP can be clinically categorized as an interstitial pneumonia, though prominent granulation tufts are seen in the airspaces. Though differences between the nonspecific interstitial pneumonia and other lips can be histopathologically clarified, the focus of clinical research on NSIP is differentiation from BOOP, or from IPF and UIP. IIP can be categorized into two groups: groups with acute or subacute lung injuries or fibrosis, such as in acute interstitial pneumonia, BOOP and nonspecific interstitial pneumonia, and groups with chronic injuries or fibrosis, such as IPF/UIP. This classification accords well with the maturity of fibrosis, CT findings, bronchoalveolar lavage fluid cell findings, and prognosis. The most critical problem is the treatment of IPF/UIP, because of its high mortality.     

具有自身免疫特征的间质性肺炎自身抗体与影像表现的相关性分析

目的总结具有自身免疫特征的间质性肺炎(IPAF)的临床特点,探讨自身抗体与影像表现的相关性。方法收集2015年1月至2018年9月于武汉大学中南医院呼吸与危重症医学科住院的符合血清学及影像学特点的IPAF病例,对其临床资料进行回顾性分析和总结。结果共收集间质性肺病患者517例,其中IPAF 78例(15.1%)。自身抗体多表现为ANA(48.7%)、抗Ro-52抗体阳性(47.4%)。胸部高分辨率CT(HRCT)分型中最常见的为非特异性间质性肺炎(NSIP)型,占62.8%。寻常型间质性肺炎(UIP)主要见于男性(P=0.019)、吸烟(P=0.004)患者,机化性肺炎(OP)型患者年龄显著小于UIP型(P=0.040)和NSIP型(P=0.002)。自身抗体表现为ANA(P<0.001)、抗Ro-52抗体(P=0.011)阳性时,HRCT分型以NSIP型多见。NSIP合并OP型患者肺通气功能表现为混合性通气功能障碍的发生率明显高于NSIP型(P=0.020);NSIP型患者弥散功能障碍的发生率显著高于UIP型(P=0.029)。结论自身抗体表现为ANA、抗Ro-52抗体阳性时,HRCT分型主要表现为NSIP型。     

The appearance of S-100 protein-positive dendritic cells and the distribution of lymphocyte subsets in idiopathic nonspecific interstitial pneumonia

Idiopathic interstitial pneumonia (IIP) is a progressive interstitial lung disease of unknown etiology. We investigated dendritic cells in idiopathic nonspecific interstitial pneumonia (NSIP) immunohistochemically, using anti-S-100 protein antibody and anti-HLA-DR antibody and also evaluated the relationship between the distribution of S-100 protein-positive dendritic cells (S- 100 DCs) and the lymphocytic subsets in the lung tissue of NSIP. Fifteen patients with the pathological diagnosis of idiopathic NSIP and six patients with usual interstitial pneumonia (UIP) were recruited into this study. Many S-100 DCs were observed in all the cases of idiopathic NSIP but S-100 DCs were not recognized in UIP cases invariably. In the mirror section method, most S-100 DCs showed a positive reaction of anti-HLA-DR antibody but a negative reaction for anti-CD1a antibody. CD8 and CD4 positive lymphocytes were infiltrated diffusely around S-100 DCs. It was demonstrated that the infiltration of CD8 positive lymphocytes predominated in the fibrosing areas and lymphoid follicles around S-100 DCs more so than CD4 positive lymphocytes.We speculate that the pathogenesis of NSIP is different from UIP and that DC and T cell-mediated immune mechanisms may play a role in the development and perpetuation of NSIP.