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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).     

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。     

老年人特发性肺纤维化的正确诊断寓于鉴别诊断之中

特发性肺纤维化（idiopathic pulmonary fibrosis,IPF）是一种病因未知的、局限于肺的特殊的慢性纤维化性间质性肺炎,外科肺活检证实其组织学为寻常型间质性肺炎（usual interstitial pneumonia,UIP）,它是特发性间质性肺炎（idiopathic interstitial pneumonia,IIP）中最常见的类型,占IIP的60%～70%。     

老年人特发性肺纤维化

特发性肺纤维化（idiopathi cpulmonary fibrosis,IPF）是组织病理学表现为普通间质性肺炎（usual interstitial pneumonia,UIP）的一种特殊的、慢性致纤维性间质性肺炎,在特发性间质性肺炎（idiopathic interstitial pneumonias,IIPs）中占47%～71%。与其他IIPs（如呼吸性细支气管炎相关间质性肺病RB-ILD、脱屑性间质性肺炎DIP、急性间质性肺炎AIP、淋巴细胞间质性肺炎LIP、     

外科肺活检在特发性间质性肺炎诊断中的应用

特发性间质性肺炎（idiopathic interstitial pneumonia，IIP）是一组原因不明的弥漫性实质性肺疾病（DPLD），包括7种临床病理类型，发病频率分别是（临床诊断／病理诊断）：特发性肺纤维化（IPF）／普通型间质性肺炎（UIP）、非特异性间质性肺炎（NSIP）／NSIP、隐源性机化性肺炎（COP）／机化性肺炎（OP）、急性间质性肺炎（AIP）／弥漫性肺泡损伤（DAD）、呼吸性细支气管炎伴间质性肺疾病（RB—ILD）／呼吸性细支气管炎（RB）、脱屑性间质性肺炎（DIP）／DIP、淋巴细胞间质性肺炎（LIP）／LIP。IIP上述临床病理类型的确诊需要肺活检病理，在很多情况下往往需要外科肺活检。小开胸肺活检的诊断率高，     

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。     

脱屑性间质性肺炎

根据组织学的特征,目前将间质性肺炎分成下列5种类型:(1)通常的间质性肺炎(Usually interstitial pneumonia,UIP);(2)脱屑性间质性肺炎(Desquamatire interstitial pneumonia,DIP);(3)伴有弥漫性肺泡损伤的阻塞性细支气管炎(Bronchiolitis obliterans with diffuse alveolar damage);(4)淋巴样间质性肺炎(Lymphoid interstitial pneumonia);(5)巨细胞间质性肺炎(Giantcell interstitial pneumonia)。其中以UIP最常见,DIP次之。DIP的名称和概念是Liebow等于1965年首先提出的,由于其病理改变、X线特征、对治疗的反应性及预后等方面均有别于其他类型的间质性肺炎,因而成为一种独立的疾病。     

特发性肺纤维化中TGF-β1诱导上皮细胞间质转化作用机制研究进展

正特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)是一种慢性、进行性、纤维化间质性肺炎,为临床最常见的一种特发性间质性肺炎(idiopathic interstitial pneumonia,IIP),其特征性病理改变类型为普通型间质性肺炎(usual interstitial pneumonia,UIP)~([1-2])。IPF病理特点为Ⅱ型肺泡上皮细胞增     

特发性肺纤维化急性加重的影像学特点

特发性肺纤维化(idiopathic pulmonary fibrosis,IPF)为特发性间质性肺炎7个亚型中最常见的类型,病理表现为普通型间质性肺炎(usual interstitial pneumonia,UIP),是一种原因不明的以肺实质的纤维化和重塑为主要特征的进展性肺部疾病。通常IPF进展缓慢,但部分患者在病程中可能出现难以预测、暴发性并且常是致命性的快速恶化,患者突然出现临床     

特发性肺间质纤维化合并肺癌的研究进展

特发性肺间质纤维化(idiopathic pulmonary fibrosis IPF)是指原因不明并以普通型间质性肺炎(usual interstitial pneumonia UIP)为特征性病理改变的一种慢性炎症性间质肺疾病,主要表现为弥漫性肺泡炎,肺泡结构紊乱,最终导致肺纤维化[1].近年来的研究证实,IPF中肺癌(IPF with lung cancer IPF-LC)的发病率明显高于普通人群,因其诊断困难,易误诊及漏诊,且治疗手段有限,是影响IPF不良预后的又一重要因素,日益受到临床及病理医师重视,本文就IPF-LC的流行病学、发病机制、临床特点及治疗预后作一综述.     

Nonspecific interstitial pneumonia and usual interstitial pneumonia: comparison of the clinicopathologic features and prognosis

Background

Nonspecific interstitial pneumonia (NSIP) has recently been proposed as a histologic type of idiopathic interstitial pneumonia (IIP), but its broad spectrum of clinicopathologic findings and variable prognosis are poorly understood. It is particularly unclear how NSIP and usual interstitial pneumonia (UIP) are related. The present study investigated the clinicopathologic features and prognosis of NSIP, and its differential diagnosis from UIP.

Methods

The clinicopathologic findings and prognosis in 21 NSIP and 18 UIP patients who underwent surgical or video-assisted thoracoscopic lung biopsy were reviewed.

Results

NSIP was more frequent in women and showed nonspecific clinical manifestations. High-resolution computed tomography (HRCT) demonstrated ground-glass, net-like, and patchy attenuation in both lungs. Semiquantitative HRCT showed a median fibrosis score of 3 (range, 0 to 7) in NSIP patients and 5 (range, 2 to 7) in UIP patients (P<0.01). On histopathologic examination, NSIP cases were heterogeneous and the findings could be categorized into cellular and fibrosing patterns. The mean age of the NSIP and UIP patients was 48 and 60 years, respectively. The frequencies of fibroblast foci, myogelosis, honeycomb lesions, and pulmonary structural destruction in NSIP and UIP patients were 16.7% and 100% (P<0.001), 22.2% and 85.7% (P<0.05), 16.7% and 92.9% (P<0.001), and 27.8% and 100% (P<0.05), respectively. The responses to glucocorticoid treatment and the prognosis were significantly greater in NSIP than those in UIP.

Conclusions

NSIP was difficult to be differentiated from UIP by general clinical manifestations, but HRCT can be helpful for this purpose. Definitive diagnosis depends on the results of surgical lung biopsy.     

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。     

Clinical impact of the radiological indeterminate for usual interstitial pneumonia pattern on the diagnosis of idiopathic pulmonary fibrosis

BackgroundIdiopathic pulmonary fibrosis (IPF) is a fatal lung disease associated with significant morbidity and mortality. The international clinical practice guidelines for the diagnosis of IPF have recently been revised.MethodsIn this single-center retrospective study conducted between June 2006 and March 2018, 27 patients with a newly classified indeterminate for usual interstitial pneumonia (UIP) pattern on high-resolution computed tomography (HRCT) who had undergone surgical lung biopsy were enrolled at the Japanese Red Cross Medical Center. Clinical and pathological characteristics and prognosis were retrospectively analyzed from patient records.ResultsOn the basis of multidisciplinary discussion (MDD), IPF was diagnosed in six patients (22%), unclassifiable interstitial pneumonia in 5 (19%), chronic hypersensitivity pneumonitis in 10 (37%), collagen vascular disease-associated interstitial lung disease in 5 (19%), and lymphoproliferative disorder in 1 (4%) patient. Ground-glass opacity, peribronchovascular distribution, upper or middle lobe distribution, mosaic attenuation, consolidation patterns, and honeycombing were found on HRCT. Histological UIP or probable UIP was observed in seven patients. The median survival time from the initial visit was 2770 days (92.3 months). There was a significant difference in survival time in the GAP stage and honeycombing on HRCT according to the log-rank test.ConclusionsPatients with an indeterminate for UIP pattern on HRCT were more likely to have non-IPF than IPF through pathological diagnosis and MDD. GAP stage and honeycombing on HRCT may be significant risk factors for all-cause mortality.     

Prognostic significance of fibroblastic foci in usual interstitial pneumonia and non‐specific interstitial pneumonia

Background and objective: Fibroblastic foci (FF) composed of an accumulation of fibroblasts or myofibroblasts may be related to the progression of pulmonary fibrosis leading to respiratory insufficiency. Several studies have shown that the number of FF is a significant prognostic factor in usual interstitial pneumonia (UIP). The purpose of the present study was to examine whether the extent of FF is related to impairment of respiratory function and prognosis in patients with biopsy‐proven fibrosing interstitial pneumonia, including UIP and fibrotic non‐specific interstitial pneumonia (fNSIP). Methods: Fifty patients with histologically confirmed interstitial pneumonia including UIP or fNSIP were investigated, and correlations between FF and pulmonary function were evaluated. FF area was calculated as the proportion of total area (%FF) and the number of FF (FF/cm²) in the whole histological specimen from each patient. Results: The UIP group showed significantly higher %FF and FF/cm² than the fNSIP group. When UIP and fNSIP patients were analysed together, the group of patients who had died (death group) revealed significantly higher %FF and FF/cm² compared with the group of survivors, and the impairment of vital capacity and diffusing capacity of carbon monoxide was correlated with %FF and FF/cm². Conclusions: FF correlated with impaired pulmonary function and may be a useful parameter to predict prognosis in patients with UIP and fNSIP.     

糖皮质激素治疗特发性非特异性间质性肺炎疗效的回顾性调查

目的 探讨糖皮质激素治疗特发性非特异性间质性肺炎(INSIP)的疗效及预后.方法 选择经临床、影像学和病理学(CRP)诊断的INSIP患者29例,根据有无纤维化分为INSIP-1组(富细胞型,9例)和INSIP-2组(混合型和纤维化型,20例),经CRP诊断的30例普通型间质性肺炎(UIP)患者作为对照组(UIP组),回顾性分析3组患者糖皮质激素治疗的疗效及随访结果,用Kaplan-Meier法估计生存曲线.结果 INSIP-1组的发病中位年龄(48.0岁)低于INSIP-2组(56.0岁)和UIP组(58.5岁),INSIP-1组的病程[(60±28)个月]长于INSIP-2组[(48±33)个月]和UIP组[(44±23)个月],但差异无统计学意义(F=1.22,P＞0.05);INSIP-1组糖皮质激素有效例数(9/9)明显高于INSIP-2组(11/20)和UIP组(2/30),差异均有统计学意义(均P＜0.05);INSIP-1组随访时间[(56±27)个月]明显长于INSIP-2组[(23±18)个月]和UIP组[(25±17)个月],显效例数(6/9)明显高于INSIP-2组(9/20)和UIP组(0/30),差异均有统计学意义(F=9.224,均P＜0.05),死亡例数(0/9)明显低于INSIP-2组(4/20)和UIP组(16/30),差异有统计学意义(均P＜0.05).结论 INSIP纤维化程度与糖皮质激素疗效和预后密切相关,糖皮质激素治疗富细胞型INSIP有较好的反应和预后,纤维化型INSIP的预后较差,明确诊断和分型具有重要的临床意义.     

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.     

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。     

Features of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome compared with idiopathic pulmonary fibrosis

BackgroundThe different characteristics of usual interstitial pneumonia in patients with primary Sjögren׳s syndrome (UIP/pSS) compared with idiopathic pulmonary fibrosis (UIP/IPF) are not fully understood. This study aimed to compare characteristics, prognosis, and treatment responses in these patients.MethodsAmong 129 consecutive patients who underwent surgical lung biopsy to diagnose diffuse lung diseases at Kanagawa Cardiovascular and Respiratory Center between 1998 and 2002, we identified 10 and 19 patients with UIP/pSS and UIP/IPF, respectively. Baseline characteristics, chest high-resolution computed tomography (HRCT) and pathological findings, and the clinical course were compared between the two groups. Responses to immunosuppressive therapy were analyzed by comparing pulmonary function and clinical status before and one year after treatment initiation.ResultsMore patients in the UIP/pSS group tended to be female and older than those in the UIP/IPF group (mean age, 68 years vs. 62 years). In addition, they more commonly exhibited enlarged mediastinal lymph nodes and bronchial wall thickening on HRCT. Pathologically, in the UIP/pSS group, interstitial inflammation, plasma cell infiltration, lymphoid follicles with germinal centers, cysts, bronchiolitis, and pleuritis were significantly more prominent, whereas smooth muscle hyperplasia and fibroblastic foci were milder (all P<0.05). The prognosis was better for UIP/pSS compared with UIP/IPF patients (P=0.01). In addition, immunosuppressive therapy provided better disease control for those with UIP/pSS (83%, 5/6) compared UIP/IPF (7%, 1/15).ConclusionThis study identified distinct clinical, radiological, and pathological characteristics of UIP/pSS compared with UIP/IPF. Immunosuppressive treatment could be a therapeutic option for UIP/pSS.     

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.     

Prognostic factors in rapidly progressive interstitial pneumonia

Background and objective: The aim of the present study was to examine clinical and other features that might allow prognostic distinctions between histological patterns in presentations with rapidly progressive interstitial pneumonia (RPIP), and to assess prognostic factors for survival. Methods: Patients with RPIP among 425 consecutive patients with diffuse lung disease, who underwent surgical lung biopsy, were studied retrospectively. The discriminatory value of clinical and investigative features for identifying disease with a better outcome was evaluated. An a priori comparison was made between diffuse alveolar damage (DAD)/usual interstitial pneumonia with DAD pattern (Group A), and organizing pneumonia/non‐specific interstitial pneumonia pattern (Group B). Results: Twenty‐eight patients (6.6%) fulfilled the criteria for RPIP. The diagnosis was Group A disease in 15 (DAD in 10, usual interstitial pneumonia with DAD in 5), and Group B disease in 13 (organizing pneumonia in 8, non‐specific interstitial pneumonia in 5). There were no significant differences in initial findings between the groups. Prognosis was significantly better for Group B patients than for Group A patients (P = 0.021). Neither BAL nor parenchymal high‐resolution CT score was indicative of therapeutic responsiveness or outcome. Distinction between Group A and Group B on the basis of disease pattern was the only significant determinant of prognosis. Conclusions: RPIP included varied histological patterns with different outcomes, and in many cases these could not be predicted using baseline clinical data. Histology was the only significant predictor of ultimate prognosis.