特发性肺间质纤维化2例

病例摘要例1：女，5岁，因咳嗽、气喘、发热50天入院。为阵发性干咳伴喘憋，活动后从夜间发作多，体温38℃左右，体检：体温37．5℃，呼吸36次／分；脉搏120次／分，消瘦，精神弱，口周、指趾端发绀，气管居中，双肺呼吸音粗，尤罗音，心音有力，肝脾不大。血红蛋白159g／L，白细胞12．6X10~9／L，中性0．72；淋巴0．28、血沉2mm／h，类风湿因子、抗核抗体、冷凝集试验、肝功能、澳抗、结核菌素试验1：2000均阴性，血氧饱和度80％，胸片：双肺纹理重，可见点片状、毛絮状密度增高影，以中下肺野为重。查胸部CT示：两肺广泛分布粗大绳索状…     

慢性过敏性肺炎引起弥漫性肺间质纤维化的病理特征

目的 观察慢性过敏性肺炎的病理形态特征,特别是其引起的肺间质纤维化的特点,并比较其与特发性肺间质纤维化的差异和鉴别.方法 选择2000年10月至2008年1月间lO例慢性过敏性肺炎患者活检组织及临床资料,活检肺组织全部取材,石蜡包埋、HE染色.结果 10例慢性过敏性肺炎患者,男性6例、女性4例,年龄23-59岁,平均47.2岁.主要表现为慢性咳嗽,气短等,病程4个月～6年不等.7例表现为普通性问质性肺炎(UIP)样的纤维化,肺组织内斑片状纤维化,病变沿胸膜下、小叶间隔分布,同时又有细支气管周边病变.7例均有纤维母细胞灶,并可见明显的细支气管周边肺泡上皮的细支气管化生和轻度细支气管炎.3例有轻度蜂窝肺.3例为非特异性间质性肺炎(NSIP)样纤维化,表现为肺泡间隔增宽,间质纤维组织和胶原纤维增生,保留肺泡结构.伴中-重细支气管炎,肺泡间隔可见较多淋巴细胞浸润.未见蜂窝肺结构.1例可见Schaumann小体.慢性过敏性肺炎的UIP样的纤维化与特发性UIP相比,除了间质中有松散的肉芽肿和多核巨细胞以外,表现为:(1)发病年龄较轻;(2)慢性过敏性肺炎CT上病变常同时有中、上肺病变,甚至以中、上肺为主,并可见磨玻璃影和马赛克征;(3)二者病变分布不同,特发性UIP的纤维化主要分布在胸膜下和沿小叶周边分布;而慢性过敏性肺炎的UIP样的纤维化常同时累及胸膜下/小叶周边和细支气管周围(小叶中心);(4)慢性过敏性肺炎的UIP样纤维化蜂窝肺相对较轻;(5)常伴有细支气管上皮化生.慢性过敏性肺炎的NSIP样的肺间质纤维化与特发性NSIP相比,除了间质中有松散的肉芽肿和多核巨细胞以外,二者在病理形态上很难区别.结论 慢性过敏性肺炎可以引起UIP样或NSIP样的间质纤维化.按照目前过敏性肺炎的诊断标准,部分表现为NSIP样的肺间质纤维化的慢性过敏性肺炎患者,有可能被误诊为NSIP,值得注意.     

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。     

肺泡Ⅱ型上皮细胞染色体脆性位点基因FHIT与肺间质纤维化的关系

目的:研究肺间质纤维化模型大鼠肺泡Ⅱ型上皮细胞染色体脆性位点基因FHIT表达的改变.方法:SD雄性大鼠随机分为假手术组、模型组及川芎嗪用药组,分别于给药7、14、28 d后提取肺泡Ⅱ型上皮细胞行体外培养,RT-PCR法检测FHIT基因mRNA表达的改变,免疫组织化学检测FHIT蛋白表达.结果:与假手术组比较,造模7d后模型组大鼠肺泡Ⅱ型上皮细胞染色体脆性位点基因FHIT mRNA及蛋白表达无变化.用药14、28 d后,FHIT基因mRNA及蛋白表达则明显下降.而川芎嗪组比模型组有显著上调.结论:在肺间质纤维化过程中存在染色体脆性位点基因FHIT的改变,川芎嗪可以一定程度上抑制这种改变从而起到防治作用.     

吸烟对大鼠肺和肺泡Ⅱ型上皮细胞间粘附分子1表达的检测

为了解吸烟肺组织和肺泡Ⅱ型上皮细胞细胞间粘附分子 1(ＩＣＡＭ 1)ｍＲＮＡ的表达 ,我们通过对不同时间吸烟大鼠肺组织和肺泡Ⅱ型上皮细胞ＩＣＡＭ 1蛋白质水平和ｍＲＮＡ水平测定来探讨吸烟致慢性阻塞性肺疾病 (ＣＯＰＤ)发病的可能途径。2 40只大鼠放入按许三林设计的大鼠被动吸烟装置内 (中华结核和呼吸杂志 1999,7:417 419)每天燃香烟 10支使大鼠形成被动吸烟模型。在吸烟 1、2、3、4、5、6月后及正常喂养的对照组大鼠杀死解剖 ,肺泡Ⅱ型上皮细胞分离、纯化和培养并留取肺组织 ,应用免疫组织化学染色、原位杂交和逆转录聚合酶链反应 (…     

血清抗环瓜氨酸肽抗体与类风湿关节炎合并肺间质纤维化的相关性分析

分析类风湿关节炎(rheumatoid arthritis,RA)患者血清中抗环瓜氨酸肽(CCP)抗体水平与类风湿关节炎患者合并肺间质纤维化的关系.选取RA患者87例,其中合并肺间质纤维化(IPF)22例,单纯RA45例,采用酶联免疫吸附实验(ELISA)检测血清抗CCP抗体滴度,免疫比浊法检测类风湿因子(RF)滴度,...     

胸膜肺母细胞瘤2例

例1,男性,51岁。因左侧胸腔积液5个月,伴胸闷、气短3天入院。查体:左下胸部叩诊呈浊音,余未见异常。肺部CT示左胸腔背侧见梭形液体密度影,局部胸膜增厚。在全麻下行左侧胸腔廓清术、纤维板剥脱术,术中见左肺下叶后方有一囊状物,大小12 cm×10 cm×6 cm,内含约500ml透明液体,囊腔内有交织的纤维束,完整切除囊壁。     

姜黄素介导Sirt1信号通路对细菌性肺炎幼鼠免疫应答及肺间质纤维化的影响

目的 研究姜黄素介导Sirt1信号通路对细菌性肺炎幼鼠免疫应答及肺间质纤维化的影响。方法 SD雄性幼鼠90只,随机数字法将大鼠分为正常组(健康大鼠)、肺炎组(肺炎模型大鼠)、姜黄素低剂量组、姜黄素中剂量组、姜黄素高剂量组,和莫西沙星组每组15只。抗体夹心法检测免疫球蛋白水平;HE染色观察病理形态;TUNEL染色测定细胞凋亡;Masson染色观察肺纤维化情况;Western blot检测Sirt1、NF-κB蛋白表达水平。结果 肺炎组幼鼠肺泡结构破坏,有炎性细胞浸润;姜黄素低剂量组与肺炎组相比无明显改善;中、高剂量组与莫西沙星组较肺炎组有所改善且以姜黄素高剂量组效果最明显。肺炎组肺组织中肺泡结构破坏,形成大量纤维化;姜黄素低剂量组与肺炎组相比无明显改善;中、高剂量组与莫西沙星组较肺炎组有所改善且以姜黄素高剂量组效果最佳。与正常组相比,肺炎组IgG、IgM、IgA、CD4⁺、Sirt1表达降低,CD8⁺、NF-κB、NF-κB p65升高(P<0.05)。在经过姜黄素干预后IgG、IgM、IgA、CD4⁺、Sirt1表...     

肺原发性肌上皮癌1例

患者男性,47岁,因“无明显诱因咯血4天”入院。咳鲜红色血,约150 ml,当时未予特殊处理,自行止血,外院胸部CT示：左下肺门影增大,左肺下叶基底段支气管闭塞,局部可见团块状软组织密度影,大小7．5 cm ×6．1 cm,可见分叶,密度不均,其内散在片状、结节样钙化影,肿块部分突入舌叶,包绕邻近支气管(图1),考虑肺癌可能性大。骨扫描正常,头颅正常,腹部CT正常,逐行手术切除术。     

肺间质纤维化

1．临床资料：患者男,45岁。活动后气促7年,进行性加重4年就诊。1998年患者无明显诱因出现活动后气短,多在体力劳动或骑自行车上坡时出现。无咳嗽、咳痰,无发热,当地医院查PPD阴性,给予抗结核治疗3年8个月（其体用药种类和药量不详）,患者症状无好转,活动耐量进行性下降。近4年患者气促进行性加重,目前只可在家中活动,步行超过5min或上3层楼即感气短明显。于2005年4月14日来本院就医。     

Smoking-related interstitial lung disease

Pulmonary diseases associated with tobacco smoking are a complex group of disorders ranging from chronic obstructive pulmonary disease (COPD) to lung cancer. Interstitial lung diseases (ILDs) have only recently been linked to smoking. The ILDs related to smoking include respiratory bronchiolitis-associated interstitial lung disease, desquamative interstitial pneumonia, and pulmonary Langerhans cell histiocytosis. The relationship of smoking with each of these entities has been largely established on the weight of epidemiologic evidence. Although they have been retained as distinct and separate conditions in various classifications of interstitial lung diseases, these 3 entities share a number of clinical, radiologic, and pathologic features suggesting that they represent a spectrum of patterns of interstitial lung disease occurring in predisposed individuals who smoke. Evaluation of histologic features, particularly in surgical lung biopsy samples, is important in making the distinction between these disorders. However, even after tissue biopsy, it may sometimes be difficult to clearly separate these entities. The importance of making the distinction between them lies in the different clinical management strategies used. Further experimental evidence, including genetic information, may be important in improving our understanding of these diseases.     

Smoking-related interstitial lung diseases

In pulmonary pathology, a wide spectrum of morphological changes is related to the consequences of smoking, and recognizing them on surgical specimens and on small transbronchial biopsies represents a challenge for the pathologist. Respiratory bronchiolitis, also referred to as smoker's bronchiolitis, is a common histologic feature found in the lung tissue of cigarette smokers. When identified as the sole histopathologic finding in the clinical setting of symptomatic interstitial lung disease, a diagnosis of respiratory bronchiolitis-interstitial lung disease is made. Since smoking is recognized to cause a variety of histologic patterns encompassing respiratory bronchiolitis, respiratory bronchiolitis-interstitial lung disease, desquamative interstitial pneumonia and pulmonary Langerhans cell hystiocytosis, smoking-related interstitial lung disease may be a useful concept to keep in mind for the pathologists. The relationship of smoking with each of these entities has been largely established on the basis of epidemiologic evidence. Although they have been retained as distinct and separate conditions in various classifications of interstitial lung diseases, these entities share a number of clinical, radiologic, and pathologic features suggesting that they represent a spectrum of patterns of interstitial lung disease occurring in predisposed individuals who smoke. Evaluation of histologic features, particularly in surgical lung biopsy samples, is important in making the distinction between these disorders. However, even after tissue biopsy, it may sometimes be difficult to clearly separate these entities. Recently, respiratory bronchiolitis-interstitial lung disease with fibrosis has been described and postulated that this is a smoking-related condition distinct from fibrotic non-specific interstitial pneumonia.     

Mediastinal and retroperitoneal fibrosis with fibrotic pulmonary nodules: a case report

We report a patient with a long history of pan-hypopituitarism treated with pituitary snuff who developed superior mediastinal fibrosis involving the visceral pericardium, retroperitoneal fibrosis, and a peculiar widespread nodular fibrosis of the lungs.     

先天性肝纤维化一例

患者男,13岁.因间断腹痛,发现肝、脾肿大7个月余,于2009年6月1日入院.腹痛无明显诱因,以脐周为主,与进食无关,便后不缓解.腹部超声:门静脉左支及右支海绵样变性,门静脉主干内径正常,血流流速稍低.腹部CT(图1,2):肝大,门脉海绵样变性,肝内胆管轻度扩张,脾大,双肾多发小囊肿.胃镜:食管静脉曲张.行脾切除、贲门周围血管离断、脾-肾静脉分流、肝活检术.     

Acute interstitial pneumonia in siblings: a case report

Acute interstitial pneumonia (AIP) is a rapidly progressive condition of unknown cause that occurs in a previously healthy individual and produces the histologic findings of diffuse alveolar damage. Since the term AIP was first introduced in 1986, there have been very few case reports of AIP in children. Here we present a case of AIP in a 3-yr-old girl whose other two siblings showed similar radiologic findings. The patient was confirmed to have AIP from autopsy showing histological findings of diffuse alveolar damage and proliferation of fibroblasts. Her 3-yr-old brother was also clinically and radiologically highly suspected as having AIP, and the other asymptomatic 8-yr-old sister was radiologically suspected as having AIP.     

Diffuse interstitial pulmonary fibrosis and lung cancer

Forty-two cases of lung cancer complicated with diffuse interstitial pulmonary fibrosis (DIPF) were selected from 13,056 autopsy cases. They were divided into primary (Group I and II) and secondary (Group III) DIPF, and histopathologic and clinicopathologic studies were made. The prevalence of lung cancer with primary DIPF was 17% (8/47) which was significantly (p less than 0.01) higher than that without DIPF (7% or 886/13,009). All eight cases of lung cancer in Group I and II were male, and 7 had cigarette-smoking history. The anatomical sites of lung cancer and DIPF were all peripheral in origin, and in 7 cases located primarily in the lower lobes (left:right = 6:1). Adenocarcinoma was the most common type. The study suggests that male DIPF patients with long clinical history should be followed thoroughly for lung cancer.