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…     

特发性间质性肺炎合并肺癌

作者就其临床经验和日本文献,对特发性间质性肺炎合并肺癌的临床、病理学检查、诊断、治疗和预防等问题做了主要的论述.     

特发性间质性肺炎诊治新进展

特发性问质性肺炎（idiopathicinterstitialpneumonia,IIP）是一组病因不明的间质性肺疾病（interstitiallungdisease,ILD）。该病表现为弥漫性肺泡炎和肺泡结构紊乱,并最终导致肺纤维化,因无特效治疗,严重威胁人类的健康11]。长期以来,IlP概念和分类比较混乱,其诊断一直是临床医师困惑的问题。2002年美国胸科学会（AmericanThoracicSociety,ATS）和欧洲呼吸学会（EuropeanRespiratorySociety,ERS）发表了IIP分类和诊断标准的国际共识意见,即IIP的ATS／ERS分类,     

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。     

蛋白质组学技术应用于特发性间质性肺炎的研究进展

蛋白质组学是后基因时代出现的一门新兴科学,它是以细胞内全部蛋白质的存在及其活动方式为研究对象,其研究目的是对机体或细胞的所有蛋白质进行鉴定和结构功能分析。其核心技术包括双向电泳、质谱分析、微阵列技术和生物信息技术。本文综述了蛋白质组学研究技术及其在特发性间质性肺炎研究应用中的最新进展,对特发性间质性肺炎的诊断和治疗提供新的方向。     

特发性间质性肺炎的分类和诊断

特发性间质性肺炎(idiopathicinterstitialpneumonia,IIP)是一组原因不明的肺间质性疾病。在过去30余年里,人们对这组疾病的临床、影像学和病理学的研究和认识在不断深入,其分类与命名亦有一个演变过程。一、特发性间质性肺炎的分类最早注意这组疾病的是Liebow等[1] ,他们从病理学角度将特发性间质性肺炎分为5种病理类型,即寻常型间质性肺炎(usualinterstitialpneumonia,UIP) ,脱屑性间质性肺炎(desquamativeinterstitialpneumonia ,DIP) ,闭塞性细支气管炎伴间质性肺炎(bronchioliticobliteranswithinterstitialpneumonia ,BIP) ,淋…     

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）,     

从特发性肺纤维化到特发性间质性肺炎:概念上的变迁

在过去四年中，特发性肺纤维化(idiopathic pulmonary fibrosis，IPF)的概念发生了一系列的变化，反映了对疾病认识的进步。只有了解了从IPF到特发性间质性肺炎(idiopathic interstitial pneumonia，IIP)在概念上的变化过程，才不会对IPF和IIP所包含的种种名词及不同内涵感到困惑。而2002年美国胸科协会(ATS)和欧洲呼吸协会(ERS)对IIP的分类发表的共识意见对疾病名称和诊断标准进行了规范化的界定^[1]。     

特发性间质性肺炎的现代概念及研究进展

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慢性炎症及纤维化所导致的异质性非特异性疾病,其中包括特发性肺纤维化（idiopathic pulmonary fibrosis, IPF）及其以外的其它间质性肺炎,发病原因不明,     

特发性间质性肺炎诊断中支气管镜的应用

目的分析评价支气管镜活检(TBLB)在特发性间质性肺炎(IIP)诊断中的应用价值。方法分析46例IIP患者的临床资料,结合其已有的胸部HRCT结果进行重新读片,并对于其相应的TBLB标本病理进行重新诊断,结合治疗效果,对比分析HRCT和TBLB的误诊和漏诊率;同时分析影响TBLB诊断价值的因素。结果对于UIP的诊断,HRCT的漏诊率为20%,误诊率为38.89%;TBLB的漏诊率为14.29%,误诊率为9.52%。结论 TBLB的漏诊率和误诊率显著低于HRCT;且当肺组织块数量≥3块时,TBLB具有较高的诊断价值;HRCT表现为非UIP者比HRCT表现为UIP更易获得有诊断价值的TBLB标本。     

老年急性间质性肺炎与特发性肺纤维化的诊治

195 3年Ｈａｍｍａｎ和Ｒｉｃｈ报道了 4例病情发展很快在半年内死亡的肺弥漫性疾病 ,以进行性加重的呼吸困难、咳嗽、伴或不伴发热的疾病。尸检显示肺泡炎症及肺纤维化 ,即以Ｈａｍｍａｎ -Ｒｉｃｈ综合征命名。在积累大量临床经验后发现Ｈａｍｍａｎ -Ｒｉｃｈ综合征的绝大部分以慢性发展为主 ,最长病程可达 10～ 15年 ,由于其病理变化主要表现为肺间质纤维化 ,遂命名为特发性肺纤维化 (ＩＰＦ)。根据发病的快慢与病程的长短分为急性型与慢性型。急性型Ｈａｍｍａｎ -Ｒｉｃｈ综合征 ,病程短半年内死亡 ,后发现其病理变化表现为弥漫性肺泡…     

Increased CD4+ T cell infiltrates in rheumatoid arthritis-associated interstitial pneumonitis compared with idiopathic interstitial pneumonitis

OBJECTIVE: To study lymphocyte markers in rheumatoid arthritis (RA)-associated interstitial pneumonitis (IP) compared with idiopathic IP. METHODS: Paraffin-embedded lung biopsy specimens from patients with RA (n = 15) and from those without RA (n = 16), all of whom had a diagnosis of either nonspecific IP or usual IP, were studied. Tissue sections from each patient were reviewed by a pathologist, who was blinded to the clinical data. Age and pulmonary function test results were similar in RA and non-RA patients. After high-temperature antigen unmasking, sections were incubated with mouse monoclonal antibodies directed against CD3, CD4, CD8, CD16, and CD20. All slides were coded, and digital images (100x magnification) of the entire tissue area were obtained. Staining was quantified using computer-assisted image analysis. RESULTS: Staining for CD4 was more prominent in patients with RA than in the non-RA comparison group (median 9.3 cells/mm(2), interquartile range [IQR] 5.5-27.3 versus 0.6 cells/mm(2), IQR 0.2-1.9; P = 0.002). CD4+ cell counts were increased in RA patients with nonspecific IP as well as in RA patients with usual IP, with no major difference between these groups. Results were similar for quantification of CD3 (P = 0.012). There was a less striking trend toward more CD8+ cells in RA patients (P = 0.27 versus those with non-RA lung disease). CONCLUSION: IP lesions in patients with RA are characterized by an increased number of CD4+ cells, as compared with that in patients with idiopathic IP. This finding suggests that CD4+ T cells are critical for the development of pulmonary manifestations in RA, and may have implications for the treatment of RA-associated lung disease.     

Nonspecific interstitial pneumonitis: a new anatomoclinical entity among idiopathic diffuse interstitial pneumonias

Nonspecific interstitial pneumonitis with fibrosis has been individualized within the group of idiopathic diffuse interstitial pneumonias by pathological criteria. It is differentiated from usual interstitial pneumonitis by the temporal uniformity of the lesions, a prominent inflammatory interstitial infiltration, and the absence of honeycombing. Clinical and functional symptoms are those of diffuse interstitial pneumonitis. An etiology may be found in about half the cases, including connective tissue disease, exposure to organic antigens, or recent acute lung injury. Computed tomography of the chest shows bilateral ground glass opacities, and alveolar opacities with a peribronchiolar or patchy distribution. Prognosis is rather good, since a majority of patients improve when treated with corticosteroids or with an association of corticosteroids and immunosuppressive drugs. These etiologic and prognostic features justify the individualization of nonspecific interstitial pneumonitis with fibrosis as a distinct clinicopathological entity.     

Chronic summer-type hypersensitivity pneumonitis initially misdiagnosed as idiopathic interstitial pneumonia

The clinical features of chronic hypersensitivity pneumonitis (HP) are similar to idiopathic interstitial pneumonias (IIPs) including idiopathic pulmonary fibrosis (IPF). We report 2 cases of chronic summer-type HP with insidious onset. They were misdiagnosed as having IIPs before referral to our hospital. Anti-trichosporon antibodies were positive in these cases. Their disease progressed due to the intermittent or continuous exposure to the antigen. Chronic summer-type HP should be included in the list of differential diagnosis of chronic interstitial lung diseases. Environmental investigation for an accurate diagnosis is important to convince the patient of the necessity to strictly avoid any future exposure to antigen.     

Increased CD4+ T cell infiltrates in rheumatoid arthritis–associated interstitial pneumonitis compared with idiopathic interstitial pneumonitis

Objective

To study lymphocyte markers in rheumatoid arthritis (RA)–associated interstitial pneumonitis (IP) compared with idiopathic IP.

Methods

Paraffin‐embedded lung biopsy specimens from patients with RA (n = 15) and from those without RA (n = 16), all of whom had a diagnosis of either nonspecific IP or usual IP, were studied. Tissue sections from each patient were reviewed by a pathologist, who was blinded to the clinical data. Age and pulmonary function test results were similar in RA and non‐RA patients. After high‐temperature antigen unmasking, sections were incubated with mouse monoclonal antibodies directed against CD3, CD4, CD8, CD16, and CD20. All slides were coded, and digital images (100× magnification) of the entire tissue area were obtained. Staining was quantified using computer‐assisted image analysis.

Results

Staining for CD4 was more prominent in patients with RA than in the non‐RA comparison group (median 9.3 cells/mm², interquartile range [IQR] 5.5–27.3 versus 0.6 cells/mm², IQR 0.2–1.9; P = 0.002). CD4+ cell counts were increased in RA patients with nonspecific IP as well as in RA patients with usual IP, with no major difference between these groups. Results were similar for quantification of CD3 (P = 0.012). There was a less striking trend toward more CD8+ cells in RA patients (P = 0.27 versus those with non‐RA lung disease).

Conclusion

IP lesions in patients with RA are characterized by an increased number of CD4+ cells, as compared with that in patients with idiopathic IP. This finding suggests that CD4+ T cells are critical for the development of pulmonary manifestations in RA, and may have implications for the treatment of RA‐associated lung disease.

     

Cephalosporin-induced interstitial pneumonitis

This report describes a patient who developed dyspnea and bilateral pulmonary infiltrates following exposure to cephradine. The role of cephradine was substantiated by rechallenge.     

Cyclosporine treatment of advanced interstitial pneumonitis associated with systemic sclerosis

A patient who had systemic sclerosis (SSc) with interstitial pneumonitis (IP) was being treated with prednisolone,d-penicillamine (D-P) and colchicine but developed progressive respiratory insufficiency. His ventilatory function showed the progression of restrictive disturbance without an obstructive one. We thought that this worsening was due to the developing IP but not bronchiolitis obliterans induced by D-P and started cyclosporine (CSA) therapy at 3 mg/kg/day. His symptoms improved after 3 months, and pulmonary function tests and blood gas analysis showed the best results after 1 year. There were no life-threatening side effects. CSA is an acceptable agent for advanced interstitial pneumonitis associated with SSc.     

Cyclosporine treatment of advanced interstitial pneumonitis associated with systemic sclerosis

Abstract

A patient who had systemic sclerosis (SSc) with interstitial pneumonitis (IP) was being treated with prednisolone,d-penicillamine (D-P) and colchicine but developed progressive respiratory insufficiency. His ventilatory function showed the progression of restrictive disturbance without an obstructive one. We thought that this worsening was due to the developing IP but not bronchiolitis obliterans induced by D-P and started cyclosporine (CSA) therapy at 3 mg/kg/day. His symptoms improved after 3 months, and pulmonary function tests and blood gas analysis showed the best results after 1 year. There were no life-threatening side effects. CSA is an acceptable agent for advanced interstitial pneumonitis associated with SSc.