非特异性间质性肺炎八例临床病理分析

目的：探讨非特异性间质性肺炎（NSIP）的病理和临床特点，方法：对8例经电视胸腔镜或小切口开胸肺活检诊断为NSIP的病例进行光镜观察和临床病理资料回顾性分析。结果：NSIP多见于女性（男：女＝1：3，2／6），平均年龄48岁，临床表现为渐进性呼吸困难，咳嗽，咳痰，双下肺闻及爆裂音，肺功能主要为限制性通气障碍，高分辨CT表现为双下肺野为主的网状，片状磨玻璃样改变，病理特征为均一的间质炎症和纤维化，4例有闭塞性细支气管炎伴机化性肺炎样改变，3例有纤维母细胞灶，1例有窝蜂肺，组织学类型，混合型6例，纤维化型2例，对皮质激素反应：显效组与有效组各4例，与纤维化型相比，大部分混合型对皮质激素有较好的反应，结论：NSIP的组织学类型与疗效有密切的关系，纤维化性的NSIP与普通型间质性肺炎在病理组织学上不易鉴别，诊断需密切联系临床。     

普通型间质性肺炎和非特异性间质性肺炎肺组织中不同细胞因子的表达及其意义

目的　研究转化生长因子 (TGF) β1、碱性成纤维细胞生长因子 (b FGF)、白细胞介素 8(IL 8)、白细胞介素 13(IL 13)、γ干扰素 (IFN γ)在普通型间质性肺炎 (UIP)和非特异性间质性肺炎(NSIP)肺组织中的分布、表达及意义。方法　经胸腔镜或开胸肺活检获取 5例UIP和 8例NSIP患者的肺组织。对照组 5例 ,来自手术切除的远离肺癌原发灶的周边肺组织。用免疫组化法半定量分析细胞因子的分布及表达。结果　TGF β1、IL 8、b FGF主要分布在肺泡上皮细胞、肺泡巨噬细胞、细支气管上皮细胞 ,UIP组表达强于NSIP组和对照组。IL 13主要分布在肺泡上皮细胞、肺泡巨噬细胞、间质单个核细胞 ,UIP、NSIP组表达无明显差异 ,但均强于对照组。IFN γ主要分布在间质单个核细胞 ,NSIP组表达强于UIP组和对照组。UIP组的IL 13/IFN γ比值为 (2 18± 0 76 ) ,NSIP组为(0 95± 0 2 8) ,对照组为 (0 91± 0 16 ) ,3组比较差异均有显著性 (P值均 <0 0 5 ) ,而NSIP组与对照组比较差异无显著性。对照组只有肺泡巨噬细胞表达上述各细胞因子。结论　TGF β1、IL 8、b FGF在UIP和NSIP患者肺组织中表达强度的不同和IL 13/IFN γ的是否平衡可能参与了UIP和NSIP不同的发病过程。     

普通性间质性肺炎的病理诊断

目的 探讨普通型间质性肺炎（ＵＩＰ）与其他类型特发性肺纤维化（ＩＰＦ）在病理上的鉴别诊断问题。方法 ＵＩＰ的尸检病例４例,复习其常规染色及组化染色切片。结果 ＵＩＰ的病理学特征是肺间质的病变轻质量不一,新老并存,既有慢性炎症改变, 进展期的肺泡隔明显纤维化及终末期的蜂窝肺和肌硬化等改变。结论 将ＵＩＰ与其他类型ＩＰＦ加以临别具有重要的临床意义,因为这些病变的预后和治疗均同。     

非特异性间质性肺炎临床-放射-病理诊断分析

目的对经肺活检诊断的非特异性间质性肺炎(NSIP)病例进行分析，探讨临床一放射一病理诊断的重要性。方法呼吸科、放射科和病理科医师对出院诊断和疑诊的9例NSIP病例的临床资料、影像特征和病理诊断进行回顾性分析，根据美国胸科学会和欧洲呼吸学会(ATS／ERS)的分类标准，重新作出一致的临床和病理诊断。结果出院诊断NSIP患7例，病理标本来自外科肺活检；疑诊NSIP患2例，病理标本来自CT引导下肺穿刺活检。回顾性病理分析发现，外科肺活检诊断的7例中5例符合ATS／ERS诊断标准，确诊为NSIP；1例主要病理特征为弥漫性支气管扩张，1例为机化性肺炎，不能诊断NSIP。经CT肺活检疑诊的2例因组织标本较小，不能进行全面病理评价，但因缺乏其他特征性病变，结合临床和影像表现，仍拟诊NSIP。全部病例的影像学表现以磨玻璃样阴影为主，均不具备特发性肺纤维化(IPF)的典型特征。确诊NSIP的5例中1例存在多肌炎／皮肌炎，临床诊断继发性NSIP；其余4例未发现潜在病因，临床诊断特发性NSIP，其中1例在诊断后3年因肺纤维化进行性加重、呼吸衰竭死亡。2例疑诊病例中1例在人院后20d死亡，1例在2年后确诊多肌炎／皮肌炎。结论NSIP的影像学表现缺乏特征性，外科肺活检是确立诊断的主要手段。NSIP的临床和病理诊断需要临床、放射和病理科医师的共同参与，而进一步寻找潜在病因是诊断过程中的重要目标。     

特发性间质性肺炎的临床诊断方法

特发性间质性肺炎（IIPs）为一组类型不同的疾病,各类型对糖皮质激素（GC）治疗的疗效反应和预后不一,故备受临床医生对诊断的关心,本病从病变的组织病理区分为：特发性肺纤维化／隐源性纤维化性肺泡炎（IPF／CFA,又称普通型间质性肺炎UIP）,非特异性间质性肺炎（NSIP）,隐源性机化性肺炎／闭塞性细支气管炎伴机化性肺炎（COP／BOOP）,急性间质性肺炎（AIP）,     

特发性非特异性间质性肺炎与普通型间质性炎疾病特征和预后的比较分析

目的 与普通型间质性肺炎(usual interstitial pneumonia,UIP)进行比较分析,探讨特发性非特异性间质性肺炎(idiopathic nonspecific interstitial pneumonia,INSIP)的疾病特征和预后以及与UIP的鉴别诊断.方法 发对经电视胸腔镜或小切口开胸肺活检诊断的21例INSIP患者和18例UIP患者的临床-影像-病理学资料及疗效、预后进行比较分析.结果 INSIP多见于40～50女性,临床表现无特异性,主要表现为活动后气促、咳嗽、咯痰、双下肺可有或无吸气相爆裂音;高分辨率CT(HRCT)表现为双肺弥漫分布的磨玻璃样淡斑片状和不规则网织状阴影,部分可有蜂窝肺.INSIP的病理特征为病变进展相对一致,按病理表现可分为细胞型、纤维化型和混合型.与UIP相比,纤维母细胞灶、肌硬化、镜下蜂窝肺和肺泡结构改建的检出率在INSIP和UIP分别是19.05%和100%(P<0.001),19.05%和88.89%(P<0.05),23.81%和94.44%(P<0.01),33.33%和100%(P<0.01).两者对糖皮质激素的反应率分别为76.19%和38.89%(P<0.01),各型INSIP的预后均明显好于UIP.结论 经 INSIP的一般临床表现差异不明显,HRCT对疑难病例的鉴别诊断有帮助,明确诊断依赖肺活检病理诊断;纤维母细胞灶、伴胶原沉积的瘢痕化和蜂窝变组成不同时相的病变共同构成诊断UIP的形态特征,也是与INSIP的鉴别要点.     

非特异性间质性肺炎1例

患者 ,男性 ,70岁 ,因咳嗽 3个月 ,活动后气短 2个月收入院。干咳 ,少许白粘痰 ,伴有间断发热 ,体温在 37～ 38℃之间。入院前 2个月活动后气短 ,登 3层楼感憋喘 ,伴心悸 ,症状有逐渐加重趋势。曾在外院静滴多种抗生素 ,效果不显著。无关节肌肉肿痛史 ,服药史及粉尘接触史。查体 :轻度紫绀 ,无杵状指 ,双肺可闻及爆裂音。血气分析 :未吸氧气静息状态 ,ＰａＯ2 6 7 5ｍｍＨｇ(1ｍｍＨｇ =0 133ｋＰａ) ,血清ＡＮＡ ,ＲＦ ,ＥＮＡ均为阴性。肺功能 :通气功能轻度限制障碍 ,弥散功能重度减退 ,ＣＯ弥散量为预计值的 4 9%。胸片 (图 1)示图 1　…     

普通型间质性肺炎的病理诊断

普通型间质性肺炎（usualinterstitial pneumonia．UIP）由Liehow于1969年首次提出．为特发性间质性肺炎（idjopa thic interstitial pneumonia．IIP）的最常见类型．又称为特发性肺纤维化（IPF），为一组原因不明，并以肺间质病变为主的疾病。该疾病目前尚无有效的治疗方法．且预后差。近年来成为临床和病理学研究的热点．现就UIP的主要临床病理特点、诊断及治疗预后的研究进展情况作一概述。     

特发性间质性肺炎基础和临床研究的几点进展

以特发性肺纤维化(IPF)为代表的特发性间质性肺炎(IIPs)一直是呼吸系统疾病诊治的难点和研究热点。近年来,国内外学者对其分类、发病机制、临床和病理诊断以及治疗等方面进行了广泛而深入的研究。现就基础和临床研究的几点进展概述如下。一、IPF发病机制研究方面的进展肺纤维化发病机制仍是众多学者研究的热点。近年来国内外学者在继续关注肺纤维化形成过程中信号传导、基质金属蛋白酶、细胞因子网络等因素的作用的同时,经研究发现,Ⅱ型肺泡上皮细胞损伤和凋亡是肺纤维化的重要早期特征。日本学者Maeyama等[1] 进行的博莱霉素致肺纤维化…     

特发性间质性肺炎临床研究的新动向

特发性间质性肺炎（idiopathic interstitial pneumonias，IIP）是病因不明的一组间质性肺疾病的总称。2002年，美国胸科学会和欧洲呼吸学会（ATS／ERS）发表的多学科国际性共识报告中，根据临床、影像学、病理的特点将其分为7个亚型。近几年，国内外学者对IIP分类本身以及IIP中最具代表性的疾病——特发性肺纤维化（IPF）／普通型间质性肺炎（usual interstitial pneumonia，UIP）进行了深入的研究并取得一定的进展，使人们感到，在本研究领域新的认识与争议并存；在IPF的药物治疗方面，困境与希望同在。     

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特发性间质性肺炎(idiopathic interstitial pneumonia, IIP)是弥漫性肺实质疾病(diffuse parenchymal lung dis ease,DPLD)中的一组疾病.它包括特发性肺纤维化 (IPF)/病理上表现为寻常型间质性肺炎(UIP),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),呼吸性细支气管炎并间质性肺疾病(RB-ILD),脱屑性间质性肺炎(DIP)和淋巴细胞性间质性肺炎(LIP)(见图1).     

Recent advances in the diagnosis and management of nonspecific interstitial pneumonia

Idiopathic interstitial pneumonia is a heterogeneous group of diseases. Recently, the importance of a detailed differentiation of nonspecific interstitial pneumonia from other idiopathic interstitial pneumonias has been demonstrated. Most critical appeared to be a proper classification based on clinical presentation, high-resolution CT findings, bronchoalveolar lavage fluid cell findings, and histopathology. This classification may guide the most appropriate therapeutic approach and has significant implications regarding prognosis. Recent advances in the diagnosis and management of nonspecific interstitial pneumonia, cellular and fibrosing variants, are discussed.     

Infliximab-induced nonspecific interstitial pneumonia

Infliximab has well-established complications including injection site and allergic reactions, cytopenias, induction of autoimmune and demyelinating diseases and malignancy, especially lymphoma. Pulmonary complications are well documented and include serious respiratory infections from tuberculosis, fungal and opportunistic pathogens. This has prompted a Food and Drug Administration black-box warning recommending close surveillance for these diseases. Nonspecific interstitial pneumonitis (NSIP) secondary to tumor necrosis factor-alpha inhibitor (TNF-alpha) therapy is less well described. Rarely, TNF-alpha inhibitor therapy has been reported to cause NSIP when used in conjunction with other immunosuppressive agents. Literature search revealed 12 independent patients with presumed infliximab-induced NSIP in 8 separate publications; all patients were on concomitant steroid sparing immunosuppressive agents, complicating cause and effect. The authors report a case in which infliximab is surmised to cause NSIP in the absence of other steroid sparing immunosuppressants in a young female with ulcerative colitis. Of importance, the patient was taking no additional steroid sparing immunomodulating agents. The diagnosis was based on clinical presentation and radiologic and histopathological data. Cessation of infliximab and high-dose steroid therapy resulted in complete resolution of the patient's presenting signs and symptoms.     

Propylthiouracil-induced nonspecific interstitial pneumonia

Propylthiouracil (PTU) is a drug used to treat hyperthyroidism. A number of adverse effects have been reported with this drug, including fever, agranulocytosis, skin rash, and vasculitis. PTU-induced interstitial pneumonia is rare--only three cases have been reported--and PTU-induced nonspecific interstitial pneumonia (NSIP) has not been reported. We report a patient who developed NSIP after taking PTU for 1 year. She developed dyspnea, cough, and mild fever lasting 1 month, and a chest CT scan showed multifocal patchy consolidation in both lungs. She underwent a surgical lung biopsy, and NSIP was confirmed pathologically. The symptoms and abnormalities seen in the chest radiograph improved after withdrawal of PTU. To our knowledge, this is the first documented case of pathologically proven PTU-induced NSIP.     

Fibroblast contractility: usual interstitial pneumonia and nonspecific interstitial pneumonia

The aim of this study was to compare the function of lung fibroblasts obtained from surgically biopsied specimens of patients with idiopathic pulmonary fibrosis/usual interstitial pneumonia (UIP; n = 5), nonspecific interstitial pneumonia (NSIP; n = 5), and normal parts of surgically resected lungs (control; n = 5). The results showed that (1) fibroblasts obtained from UIP showed increased contractility compared with those obtained from NSIP or controls (UIP, 72.7 +/- 6.21%; NSIP, 32.8 +/- 5.46; controls, 28.5 +/- 3.51, p < 0.01 in UIP versus NSIP or control); (2) this increase in contractility was consistent with enhanced F-actin content in fibroblasts; (3) conditioned media from UIP fibroblast cultures enhanced control fibroblast contractility, whereas those obtained from NSIP or controls did not; (4) the 180 and 25 kD products representing the contractility in conditioned media were identified as fibronectin (ED-A domain) and TGF-beta1 by immunoblots, respectively; (5) the UIP-conditioned media contained higher amounts of fibronectin or TGF-beta 1 (fibronectin: UIP 289 +/- 47.1 ng/ml, NSIP 121 +/- 23.0, control 118 +/- 16.0; TGF-beta1: UIP 798 +/- 119 pg/ml, NSIP 246 +/- 69.1, control 247 +/- 53.6, p < 0.01 in UIP versus NSIP or control); () the contractility positively correlated with the amount of either fibronectin (r = 0.867, p < 0.001, n = 15) or TGF-beta 1 (r = 0.939, p < 0.001, n = 15), respectively. Thus, UIP fibroblasts showed greater contractility than did NSIP fibroblasts and up-regulated control fibroblasts.     

Idiopathic interstitial pneumonias: usual interstitial pneumonia versus nonspecific interstitial pneumonia

Diffuse parenchymal lung diseases (DPLDs) are a group of disorders that involve the space between the epithelial and endothelial basement membranes. Recent guidelines for the classification of DPLDs recommended separating patients into several categories, including (1) DPLDs of known cause, (2) granulomatous DPLDs, (3) rare DPLDs with well-defined clinicopathologic features, and (4) the idiopathic interstitial pneumonias (IIPs). The IIPs are further subdivided into categories that include usual interstitial pneumonia (idiopathic pulmonary fibrosis [IPF] if the usual interstitial pneumonia is idiopathic in origin) and nonspecific interstitial pneumonia. In numerous cohorts, IPF has been associated with impaired prognosis compared with nonspecific interstitial pneumonia, except in the setting of markedly impaired physiology at presentation. It is therefore imperative for the health care provider to rapidly assess the likelihood of a patient having IPF. The diagnostic approach has been refined over the past several years, with much clearer recommendations addressing the optimal clinical, radiologic, and laboratory assessments of patients with IIPs. Similarly, therapeutic interventions have rapidly evolved, with less emphasis on standard immunosuppression and increasing focus on interventions targeting fibroproliferation. Numerous therapeutic trials have recently been completed, are ongoing, or are soon to begin patient recruitment. A multicenter, National Institutes of Health-funded clinical research network has been convened to target novel therapeutic approaches in well-designed controlled trials. The next few years will be an exciting time in the evaluation and treatment of DPLDs as increasing clinically relevant biological information is translated to novel diagnostic and therapeutic approaches.     

BAL findings in idiopathic nonspecific interstitial pneumonia and usual interstitial pneumonia.

Idiopathic pulmonary fibrosis (IPF), which has the histological pattern of usual interstitial pneumonia (UIP), is a progressive interstitial lung disease with a poor prognosis. Idiopathic interstitial pneumonias with a histological pattern of nonspecific interstitial pneumonia (NSIP) have a better prognosis than UIP, and may present with a clinical picture identical to IPF. The authors hypothesised that bronchoalveolar lavage (BAL) findings may distinguish between UIP and NSIP, and have prognostic value within disease subgroups. BAL findings were studied retrospectively in 54 patients with histologically proven (surgical biopsy) idiopathic UIP (n=35) or fibrotic NSIP (n=19), all presenting clinically as IPF. These findings were also compared with the BAL profile of patients with other categories of idiopathic interstitial pneumonias. BAL total and differential cell counts did not differ between the two groups. Survival was better in NSIP. In neither group were BAL findings predictive of survival or changes in lung function at 1 yr, even after adjustment for disease severity, smoking and treatment. BAL differential counts in fibrotic NSIP differed from respiratory bronchiolitis-associated interstitial lung disease, but not from desquamative interstitial pneumonia or cellular NSIP. The authors conclude that bronchoalveolar lavage findings do not discriminate between usual interstitial pneumonia and nonspecific interstitial pneumonia in patients presenting with clinical features of idiopathic pulmonary fibrosis, and have no prognostic value, once the distinction between the two has been made histologically.     

特发性肺纤维化与非特异性间质性肺炎的非创伤性鉴别诊断方程

目的 为提高临床非创伤性区分特发性肺纤维化(IPF)与非特异性间质性肺炎(NSIP)的水平,建立IPF与NSIP的非创伤性鉴别诊断方程.方法 选病理确诊的14例IPF和18例NSIP患者为研究对象,记录患者临床资料、胸部高分辨率CT评分、支气管肺泡灌洗等结果.将各个变量分别赋值,筛选,建立方程,确定临界值.结果 (1)IPF组患者比NSIP组年龄偏大,男性较多,吸烟者较多.(2)与NSIP组比,IPF组患者胸部高分辨率CT评分网格影、蜂窝影评分高,磨玻璃影评分低.(3)IPF组淋巴细胞占支气管肺泡灌洗液中细胞总数大于20%低于NSIP组.(4)建立方程:Y=0.9+0.123x1-0.045x2+0.009x3+0.033x4(x1、2、3、4分别为影像学显示蜂窝影、影像学显示磨玻璃影、年龄、影像学显示网格影),方程临界值为1.5.结论 回归方程y=0.9+0.123x1-0.045x2+0.009x3+0.033x4可协助临床医生区分IPF与NSIP.