慢性外源性过敏性肺泡炎七例病理诊断和临床分析

目的 探讨慢性外源性过敏性肺泡炎的病理诊断及其诊断思路.方法 收集首都医科大学附属北京朝阳医院2006年10月至2009年3月收治的、经病理确诊且有完整临床资料的慢性外源性过敏性肺泡炎7例,对其病理组织学形态和临床资料进行综合分析,并对文献进行复习.结果 7例慢性外源性过敏性肺泡炎患者,男性4例,女性3例,年龄30～65岁,平均年龄48岁,均表现为慢性起病,5例因近期加重入院,其中4例有刺激性气体或粉尘、宠物接触史;2例常见过敏原检测阳性;胸部高分辨CT显示小叶中心性小结节影及纤维条索影、磨玻璃影和小叶间隔增厚;支气管肺泡灌洗液淋巴细胞数量增高,CD4+/CD8+细胞比值＜1;肺功能检查均显示肺活量降低、肺总量降低、呼吸总气道阻力增高及周边气道阻力增高为主.5例行开胸肺活检,2例行肺移植手术.病理组织学均表现为:细支气管周围慢性炎及间质纤维化,不典型非干酪性上皮样肉芽肿,支气管腔内可见上皮样吞噬细胞渗出和新生的纤维息肉样组织形成;5例开胸肺活检的病例支气管腔内可见中性粒细胞渗出,5例均确诊为慢性外源性过敏性肺泡炎伴急性发作;2例慢性外源性过敏性肺泡炎终末纤维化期行肺移植手术.结论 慢性外源性过敏性肺泡炎组织病理学表现具有一定的诊断特征,但与其他诸多弥漫性肺实质性疾病具有相似的病理改变,须结合病史、影像学等临床信息综合分析,从而明确诊断,进行有效治疗.     

过敏性肺炎:饲鸽者肺的病因、临床特征与发病机制

过敏性肺炎(Hypersensitivity Pneumonifis，HP)又称外源性变应性肺泡炎(extrinsic allergic alveolitis)。它包括了一组由于机体反应性增高和反复吸入某种抗原所致的远端支气管和肺泡发生的慢性肉芽肿样炎症，这些抗原包括哺乳类和鸟类的蛋白、真菌、嗜热性细菌以及某些低分子量化合物等…。以往以职业性暴露所致的发病较为多见，近年来暴露于居住环境的宠物鸟(如鸽子和长尾小鹦鹉)、污染的空气     

吸烟相关性肺间质纤维化1例

吸烟相关性肺间质纤维化是吸烟引起的间质性肺疾病, 相关病例报道较少。现报道1例发生于62岁男性的吸烟相关性肺间质纤维化。CT显示胸膜下呈过度充气改变, 可见薄壁囊肿。病灶镜下可见肺泡间隔明显增宽, 增宽的肺泡间隔由致密透明样变的胶原组成, 其内相对无细胞成分。病变主要分布于胸膜下肺实质内, 深部肺实质中病变具有小叶中心性分布的特点。     

外科肺活检病理诊断过敏性肺炎47例临床病理学分析

目的:分析外科肺活检病理诊断过敏性肺炎（hypersensitivity pneumonia,HP）病例的临床病理特征及免疫组织化学特点,提高对HP的诊断水平。方法:收集中国医学科学院 北京协和医学院 北京协和医院2000年1月至2020年6月近20年外科肺活检病理诊断HP病例47例的临床病理资料,通过光镜及免疫组织化...     

关于间质性肺疾病的病理诊断

间质性肺疾病是呼吸内科疾病的一组十分重要的疾病,它包括病因已知和未知的200多种疾病,是肺非肿瘤性病变的一个重要组成部分。随着分子生物学、免疫学、细胞生物学、影像学的发展,以及经支气管肺活检,特别是开胸肺活检和电视胸腔镜下肺活检的开展,给病理学诊断与研究提供了资料,能     

慢性阻塞性肺疾病合并肺间质纤维化的临床诊疗分析

目的：探讨慢性阻塞性肺疾病合并肺间质纤维化的临床诊疗方式。方法对我院2012年6月~2014年4月收治的40例慢性阻塞性肺疾病合并肺间质纤维化患者的临床资料进行回顾性分析,对患者的临床表现、气血检查结果、CT见过及肺功能改变等进行观察。结果慢性阻塞性肺疾病合并肺间质纤维化的临床表现综合了上述两种疾病,肺功能障碍主要表现为混合型呼吸功能障碍,CT及高分辨CT、X线胸片检查结果兼有两种疾病的特点。结论慢性阻塞性肺疾病合并肺间质纤维化兼有两种疾病的特征,CT及X线胸片是诊断该疾病的重要手段,及早确诊及治疗对提高患者的生存质量具有重要意义。     

胺碘酮致肺损伤的临床病理分析

目的探讨胺碘酮致肺损伤的临床、病理和影像学特征。方法回顾性分析3例胺碘酮致肺损伤临床表现、影像学及病理表现、治疗及预后。结果3例男性患者,年龄35～64岁,就诊时病程20d～3年。均有咳嗽,2例有气短及肺部爆裂音。双肺片状浸润影、磨玻璃样变、弥漫索条及网格状改变为胸部CT主要表现。病理表现为富细胞性间质性肺炎改变;肺泡腔内见巨噬细胞聚集,部分巨噬细胞胞质呈细空泡状;肺泡Ⅱ型上皮细胞增生;1例可见机化性肺炎表现。结论胺碘酮致肺损伤病理表现有一定的特点,诊断应结合临床及影像综合考虑。     

博莱霉素A5诱发大鼠肺间质纤维化的实验病理研究

用博莱霉素A_5诱发大鼠肺间质纤维化,动态观察其病理形态及肺间质内Ⅰ、Ⅲ型胶原的变化,所用方法包括光镜、电镜及间接免疫荧光法。结果表明,实验第1周内动物以肺泡炎病变为主并有肺泡“腔内芽”形成,第2周起则以间质纤维化病变为主;Ⅰ、Ⅲ型胶原显著增多分别主要发生于实验晚期和早期。本研究提示,“腔内芽”可能参与了纤维化的形成过程,该过程中Ⅰ、Ⅲ型胶原呈动态变化。     

Pathological differentiation of chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia

Takemura T, Akashi T, Kamiya H, Ikushima S, Ando T, Oritsu M, Sawahata M & Ogura T
(2012) Histopathology
Pathological differentiation of chronic hypersensitivity pneumonitis from idiopathic pulmonary fibrosis/usual interstitial pneumonia Aims: To evaluate the histological characteristics differentiating chronic hypersensitivity pneumonitis (chronic HP) with a usual interstitial pneumonia (UIP)‐like pattern from idiopathic pulmonary fibrosis (IPF)/UIP. Methods and results: Surgical lung biopsy specimens from 22 patients with chronic HP diagnosed as having a UIP‐like pattern upon histological examination and 13 patients with IPF/UIP were examined and the incidences of bronchiolitis, perilobular fibrosis, centrilobular fibrosis, bridging fibrosis, organizing pneumonia, fibroblastic foci, honeycombing, granulomas, giant cells, lymphocytic alveolitis and lymphoid follicles were compared. Bronchiolitis, centrilobular fibrosis, bridging fibrosis, organizing pneumonia, granulomas, giant cells and lymphocytic alveolitis were significantly more frequent among patients with chronic HP than among patients with IPF (all P < 0.01). Conclusions: Centrilobular fibrosis, bridging fibrosis and organizing pneumonia, in addition to bronchiolitis, granulomas and giant cells, are characteristic features of chronic HP with a UIP‐like pattern. These features are therefore important in differentiating chronic HP from IPF/UIP, as management strategies differ for the two disorders.     

Ultrastructural study on human lung in alveolitis versus pulmonary fibrosis

Summary Lung specimens of 21 patients with diffuse interstitial lung disease were examined. The present ultrastructural study outlines the topography and distribution of inflammatory changes in the interstitium, endothelium, and in pneumocytes and phagocytes. Alveolitis is characterized by marked regenerative activity of type II pneumocytes (cuboid metaplasia), intraluminal macrophage accumulation, endothelial swelling, multilamination of the endothelial basement membrane, pericapillary edema, and primarily by cellular infiltrates in the interstitial space. The most prominent feature of the interstitium in pulmonary fibrosis is the lack of immunoinflammatory cells. In some areas there is a marked absence of alveolar lumen while only a small number of macrophages are present in the remaining alveolar lumen. Most of the capillaries in the fibrous septum have been destroyed. Ultrastructural studies of lung biopsies in patients with diffuse interstitial lung disease allow the differentiation between alveolitis and pulmonary fibrosis and thus contribute to a therapeutic decision.     

普通型间质性肺炎的临床病理特征及其与特发性非特异性间质性肺炎的鉴别诊断

目的 探讨普通型间质性肺炎(UIP)的临床病理特征以及与特发性非特异性间质性肺炎(INSIP)的鉴别诊断。方法 对15例经电视胸腔镜或小切口开胸肺活检诊断为UIP的病例进行光镜观察和临床病理资料回顾性分析,治疗后随访,与11例病理诊断的INSIP进行比较分析。结果UIP多见于50岁以上的男性,临床主要表现为活动后气促、咳嗽咳痰、双下肺闻及吸气相爆裂音;高分辨CT表现为以中下肺和胸膜下为主的片状和网状阴影,8例有蜂窝肺。UIP的病理特征为病变进展不一致,间质的炎症、纤维化和蜂窝变与正常肺组织呈交替分布,轻重不一。纤维母细胞灶、肌硬化、镜下蜂窝肺、弥漫胶原沉积和肺泡结构改建的检出情况在UIP和INSIP分别是15/15和3/11(P<0.001)、12/15和4/11(P<0.05)、13/15和3/11(P<0.01)、15/15和6/11(P<0.01)、15/15和5/11(P<0.01)。两者对糖皮质激素的反应率分别为3/15和8/11(P<0.05)。结论 UIP和INSIP的一般临床表现差异不明显,高分辨CT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。     

普通型间质性肺炎的形态学表现和病理诊断

目的 探讨普通型间质性肺炎(UIP)的病理形态特点,以及开胸肺活组织检查的意义和适应证。方法 对5例UIP(尸解1例和肺活检4例)的HE及组织化学染色的病理形态、以及临床、影像学和预后的资料进行分析。结果 其病理表现有肺泡间质炎、成纤维细胞灶、间质纤维化和蜂窝肺,并伴细支气管上皮化生和Ⅱ型细胞增生,以及灶性肺泡内巨细胞聚集。上述改变为不均匀的分布。结论 UIP的病理特点为低倍镜下的形态不一致性,并且为新老病变共存。对于影像表现不典型和能耐受开胸手术的患者,开胸肺活组织检查的病理诊断是必要的。但UIP的病理诊断过程要密切结合临床和影像学的检查。     

An unusual perilobular pattern of pulmonary interstitial fibrosis associated with Crohn's disease

Pulmonary pathology associated with inflammatory bowel disease is rare, and seldom biopsied. In this report, we describe a case of pulmonary interstitial fibrosis associated with Crohn's disease. Biopsy showed a unique pattern, with cellular fibroblastic proliferation and fibrosis affecting interlobular septa, spreading into the parenchymal interstitium from the periphery, with associated lymphoid aggregates and thickening of arterial adventitia.     

Silica-induced pulmonary fibrosis involves the reaction of particles with interstitial rather than alveolar macrophages

Macrophage-derived products have been implicated in fibroblast stimulation following particle deposition in the lung. To assess the role of macrophages in the alveolus versus those in the interstitium in the induction of pulmonary fibrosis, we compared the pulmonary response to silica when phagocytosis occurred predominantly in each of these compartments. One group of mice received intratracheal silica which was phagocytosed largely by alveolar macrophages (AM). A second group was exposed to whole body irradiation prior to receiving the same dose of silica. This prevented the usual efflux of PMN and monocytes into the air sacs, allowing passage of silica particles across the alveolar epithelium to reach the interstitial macrophages (IM). In the irradiation plus silica group, many large interstitial granulomas were formed at 2-4 weeks, and collagen levels were significantly greater than in all other groups at 16 weeks. More silica was found in a lung tissue residue and in lymph nodes of these animals. Pulmonary fibrosis was limited to interstitial areas where there was a high level of retained silica, whereas peripheral regions of the lung, where free AM containing silica were found, did not show fibrosis of the alveolar walls. The results suggest that factors secreted by IM in response to silica are more effective in stimulating fibrogenesis than secretions made by the AM into the alveolar space.     

Interstitial associations of cells lining air spaces in human pulmonary fibrosis

Summary An ultrastructural study of the cells that line air spaces in human pulmonary fibrosis is reported. Intimate associations between these cells and cellular elements in the interstitium were consistently found in biopsies from 25 cases. Cytoplasmic extensions of cuboidal pneumocytes protruded through discontinuities in the subjacent basement membrane. Attenuated cells having structural properties of fibroblasts were situated on connective tissue that formed the walls of numerous air spaces. In this situation, a basement membrane was not demonstrable. These heretofore undescribed features suggest a dynamic interaction between certain mesenchymal and epithelial elements in the fibrotic lung.This study is from a Specialized Center of Research (SCOR) in pulmonary disease supported by U.S. Public Health Service Grant No. HL-14212, from the National Heart and Lung Institute     

Nonspecific interstitial pneumonia: pathologic features and clinical implications

Nonspecific interstitial pneumonia (NSIP) is a form of chronic interstitial pneumonia that should be separated from the other idiopathic interstitial pneumonias, including most importantly, usual interstitial pneumonia (UIP). Diagnosis is predicated on identification of characteristic findings in a surgical lung biopsy in the appropriate clinical and radiological context. Affected patients may have a variety of underlying or associated conditions, although most have a form of idiopathic lung disease associated with a more favorable prognosis than UIP/idiopathic pulmonary fibrosis (IPF). Keys to distinguishing NSIP from UIP include absence of heterogeneous lung involvement, architectural distortion in the form of fibrotic scarring and/or honeycomb change, and fibroblast foci in NSIP.