成人肺朗格汉斯细胞组织细胞增多症五例临床分析

目的 探讨成人肺朗格汉斯细胞组织细胞增多症(PLCH)患者的临床、影像、肺功能和组织病理特点.方法 回顾性分析2006年6月至2007年10月北京朝阳医院收治的5例PLCH患者的临床资料.结果 5例均为男性吸烟者,以肺部损害为主,其中2例以自发性气胸为首发症状,1例为肺脏终末期病变合并肺动脉高压.胸部高分辨率CT示大小不一的多发囊腔阴影,病变以双上肺为著,终末期病例囊腔影遍及全肺.5例均经外科肺活检获得组织病理学资料,显示囊腔样改变和朗格汉斯细胞聚集(免疫组织化学CD1a和S-100染色阳性).肺功能检查示V50占预计值%为53.6%～77.6%,V25占预计值%为38.5%～70.5%,V50和V25的降低与组织病理学所见小气道受累相符合.治疗以戒烟为主,疾病有自然缓解倾向.结论 PLCH是朗格汉斯细胞组织细胞增多症的一种特殊类型,主要发生于吸烟者,多呈良性经过.     

肺朗格汉斯细胞组织细胞增多症合并肺动脉高压

目的 探讨肺朗格汉斯细胞组织细胞增多症(PLCH)合并肺动脉高压(PH)的临床表现,以提高对本病的认识.方法 回顾性分析2006年6月至2011年6月首都医科大学附属北京朝阳医院呼吸与危重症医学科收治的11例PLCH患者的临床资料.结果 11例PLCH患者中4例合并PH(36％),PLCH-PH临床症状较无PH者重,表现为Borg呼吸困难评分明显增加,杵状指,心功能(NYHA)达Ⅲ～Ⅳ级,并出现右心衰竭体征.胸部HRCT以双上中肺野弥漫囊腔样损害为主,并可见肺动脉增宽,右心增大.肺一氧化碳弥散量及动脉血氧分压也显著降低,并出现呼吸衰竭.彩色多普勒超声心动图示肺动脉收缩压升高.肺活检病理除朗格汉斯细胞浸润及囊腔样改变外,还可见肺小血管管腔狭窄及毛细血管扩张.治疗以氧疗、对症缓解症状为主,3例接受激素或联合免疫抑制剂治疗未见明显效果;4例PLCH-PH,仅1例随访1年病情稳定.结论 PH是PLCH比较常见的并发症,是PLCH病情进展、预后不良的标志;对于PLCH患者应注意PH的早期评估与预防.     

被动吸烟对大鼠血清和肺组织中白细胞介素17表达的影响

目的观察被动吸烟和戒烟对大鼠被动吸烟模型血清和肺组织中白细胞介素(IL)-17变化的影响。方法选择健康雄性Wistar大鼠50只,随机分为5组,分别为正常对照组、被动吸烟4 w、8 w、12 w组和戒烟组。HE染色观察肺组织的病理改变,采用ELISA方法检测血清中IL-17含量,免疫组织化学染色方法观察肺组织中IL-17的表达。结果被动吸烟可导致肺组织炎症反应和肺气肿改变;被动吸烟组和戒烟组大鼠血清IL-17含量较正常对照组升高,肺组织中IL-17表达增强;随着被动吸烟时间的延长,血清中IL-17含量逐渐增加,肺组织中IL-17表达逐渐增加;戒烟组较被动吸烟12 w组血清IL-17含量降低,肺组织中IL-17表达减少。结论被动吸烟可引起肺部炎症,导致血清及肺组织IL-17表达增加,IL-17可能参与了吸烟相关性肺损伤的发生。     

不同吸烟量对大鼠气道上皮细胞中细胞问黏附分子1表达的影响

目的 通过研究不同吸烟量、不同吸烟持续时间吸烟及戒烟对大鼠气道上皮细胞中细胞间黏附分子1(intercellular adhesion molecule-1,ICAM-1)mRNA及其蛋白水平表达的影响,探讨吸烟和戒烟与慢性阻塞性肺疾病气道炎症的关系.方法 雄性Wistar大鼠40只,随机分为对照组、长期大量吸烟组、长期小量吸烟组、短期大量吸烟组、长期大量吸烟后戒烟组,每组各8只,用原位杂交法及免疫组织化学法检测ICAM-1 mRNA及其蛋白在各组大鼠气道上皮细胞中的表达.结果 与对照组ICAM-1 mRNA及其蛋白(1.45±0.98,8.83±0.77)水平比较,长期大量吸烟组(6.93±1.44,19.22±0.22)、短期大量吸烟组(2.92±0.67,12.91±1.31)、长期小量吸烟组(4.76±0.68,14.03±2.39)、长期大量吸烟后戒烟组(4.84±0.94,14.95±1.82)表达均明显升高,以长期大量吸烟组最为显著,差异均具有统计学意义(P＜0.05).与长期大量吸烟组相比,长期小量吸烟组、短期大量吸烟组、长期大量吸烟后戒烟组ICAM-1 mRNA及其蛋白水平均低于长期大量吸烟组,差异均具有统计学意义(P＜0.05).结论 吸烟引起大鼠气道上皮细胞ICAM-1 mRNA及其蛋白的表达水平升高,并随吸烟时间和量的增加其表达增加,戒烟后其表达可下降.提示戒烟可减轻气道炎症,是预防慢性阻塞性肺疾病的有效措施.     

护理宣教对急性心肌梗死女性吸烟患者戒烟成功及预后的影响

目的:探讨院内护理宣教对我国急性心肌梗死患者中女性吸烟患者戒烟效果及临床预后的影响。方法:纳入并分析了自2015年1月至2017年1月,就诊于我院心内科患有急性心肌梗死的女性患者2 619例。按照患者吸烟状态将人群分为两组:吸烟组(入院时仍然吸烟者和已戒烟者)及非吸烟组(从未吸烟者)。比较两组患者基线资料。吸烟患者随机分为"干预组"和"对照组",分别接受"5 A"戒烟干预方案和常规护理。观察戒烟成功率并比较1年后戒烟成功的患者与戒烟未成功患者的预后。结果:在患有急性心肌梗死的女性患者中,吸烟者共计414(15.8%)例。相比于非吸烟者,吸烟者更年轻(66.2vs.70.3)岁,两组有高血压者(241vs.1 095;58.2%vs. 49.7%)、糖尿病(103 vs. 331;24.9%vs. 15.0%)、心力衰竭病史(18vs. 55;4.3%vs. 2.5%)比例更高。经戒烟护理宣教后随访1年,有161例(38.9%)戒烟成功。相比于未戒烟者,戒烟成功患者全因死亡率(4.3%vs. 8.1%)及再住院率(3.1%vs. 11.9%)更低。结论:在女性急性心肌梗死人群中,与非吸烟者相比,吸烟者合并其他心血管疾病危险因素比例更高。戒烟护理宣教对患者戒烟有着重要意义,对改善预后有着重要价值。     

吸烟所致肺气肿模型大鼠戒烟后肺病理及炎性介质的变化

目的 探讨吸烟所致肺气肿模型大鼠在戒烟后肺组织病理及炎性介质表达变化规律.方法 选用Wistar雄性大鼠80只,随机分为非吸烟正常对照组及吸烟所致肺气肿模型戒烟0 d、1 w、2 w、4 w、6 w、8 w、12 w组.采用酶联免疫吸附方法测定各组大鼠血清中IL-8的蛋白质含量,S-P免疫组化学方法检测肺组织NF-кB p65的表达,并光镜观察HE染色切片、对大鼠气道炎症进行病理学评分.结果 肺气肿大鼠模型成功复制:肺组织HE染色切片,光镜下观察,其病理变化与人类肺气肿相似;肺气肿各时相戒烟组大鼠血清IL-8浓度、肺组织NF-кB的表达及病理评分较未吸烟正常对照组明显升高(P<0.05);肺气肿各相邻时相戒烟组间比较,各检测指标有上升趋势,戒烟12 w组大鼠血清中IL-8的蛋白质含量、肺组织NF-кB p65 的表达、气道炎症的病理学评分均较戒烟0天组明显升高(P<0.05).结论 吸烟所致肺气肿模型大鼠形成后,其气道炎症在戒烟后12周内仍可见持续加重.     

高分辨率CT及积分在结缔组织病并发间质性肺炎及预后中的价值

目的 比较不同结缔组织病(CTD)并发间质性肺炎(ILD)的肺高分辨率CT(HRCT)影像学特征,探讨肺HRCT积分对判断患者预后的预测价值.方法 回顾性收集117例各类CTD 临床资料和影像学资料以及随访结果,对肺HRCT病变进行评分,研究肺HRCT积分与患者预后的关系.结果 117例CTD患者中有95例在肺HRCT上显示有ILD,其中,系统性红斑狼疮(SLE)34%,类风湿关节炎(RA)31%,多发性肌炎/皮肌炎(PM/DM)9%,成人斯蒂尔病(AOSD)9%,干燥综合征(SS)5%,系统性硬化症(SSc)7%,混合性结缔组织病(MCTD)5%.CTD并发ILD肺HRCT最常见的表现是小叶间隔增厚,其次为毛玻璃样变,之后依次为不规则线状影或胸膜下线、网格影、支气管血管束增厚、马赛克灌注、蜂窝肺、薄壁囊肿、结节灶、大片实变病变、肺大泡.AOSD和SSc患者纤维化积分显著高于渗出积分(P＜0.05);CTD并发ILD患者5年生存率为48.5%(95%可信区间为22.4%～67.9%),纤维化积分≥2分是预测患者预后不良的危险因素(P=0.032),其敏感性为82%,特异性为87%.结论 不同CTD并发的ILD有不同的影像学特征,肺HRCT可以较好地发现并鉴别,肺HRCT纤维化积分可以判断患者的预后.     

吸烟与肺朗格汉斯细胞组织细胞增生症

肺朗格汉斯细胞组织细胞增生症的发生与吸烟有关.烟草糖蛋白可引起一系列细胞和细胞因子病理变化.戒烟可使部分患者病情稳定,改善影像学变化,甚至可使疾病(包括肺外病变)痊愈.     

戒烟对吸烟大鼠肺组织MCP-1和TGF-β的影响

目的 :研究戒烟对吸烟大鼠肺气肿模型肺组织中的单核细胞趋化蛋白 1(MCP 1)和转化细胞生长因子 β(TGF β)的影响。 方法 :建立吸烟肺气肿大鼠模型。 6 0只雄性Wistar大鼠 ,随机分为对照组4个月组和 6个月组 ,吸烟组 4个月组和 6个月组以及吸烟 4个月和 6个月后戒烟组 ,每组 10只。用免疫组化法观察MCP 1和TGF β在肺内的表达 ,用酶联免疫吸附测定法 (ELISA)观察MCP 1在模型肺泡灌洗液 (BALF)中的表达 ,及测定各组肺组织中溶胶原活性和总羟脯氨酸的含量。结果 :与对照组相比 ,吸烟 4个月组肺组织MCP 1和TGF β的表达高于吸烟 6个月组 ,吸烟 4个月后戒烟组低于吸烟 6个月后戒烟组 ;各组BALF中MCP 1差异无统计学意义 ;各组各时段溶胶原激活活性均比自发活性高。结论 :戒烟影响MCP 1和TGF β在吸烟大鼠肺气肿模型肺组织中的表达 ,可作为肺气肿防治的有效措施。     

吸烟及戒烟对冠状动脉旁路移植术后早期结果的影响

目的:研究吸烟及戒烟对冠状动脉旁路移植术(CABG)后早期结果的影响.方法:回顾性分析我院2004-01至2005-12行CABG 2 570例患者资料,其中有吸烟情况记录者共2 486例,按照术前吸烟情况分为吸烟组(1 457例)和不吸烟组(1 029例),其中吸烟组根据术前1个月是否戒烟分为戒烟者(409例)及未戒烟者(1 048例),采用Logistic多因素回归分析吸烟及戒烟与住院期间术后早期并发症及死亡率的关系.结果:与不吸烟组相比,吸烟组接受CABG时年龄较小,男性患者占比例更高,体重指数较高,心肌梗死史高,合并慢性阻塞性肺病史高,较少合并高血压病及高脂血症,合并二尖瓣反流史低,射血分数低,较少应用体外循环,差异均有统计学意义(P<0.001-0.05).吸烟组与不吸烟组比较术后肺部并发症发生率高(3.2%vs 1.6%),呼吸机辅助时间增加(8 h vs 6 h),差异均有统计学意义(P均<0.05).多因素回归分析表明吸烟组(包括术前戒烟和未戒烟患者)发生肺部并发症的风险为不吸烟组的1.91倍(95%可信区间1.03-3.56);未戒烟者发生肺部并发症的风险为不吸烟组的2.30倍(95%可信区间1.19-4.46),差异均有统计学意义(P均<0.05);其他术后早期并发症的发生吸烟组与不吸烟组间比较差异均无统计学意义(P均>0.05).结论:吸烟组患者术后肺部并发症明显增加,呼吸机辅助时间延长,术前未戒烟患者术后并发症显著增加.应鼓励患者及早戒烟,戒烟有助于减少CABG后并发症的发生.     

Incipient stage of pulmonary Langerhans-cell histiocytosis complicated with pulmonary tuberculosis was examined by high-resolution computed tomography

A 29-year-old man with a smoking history of 20 pack years was diagnosed with tuberculosis because Mycobacterium tuberculosis was detected in his gastric juice. His chest radiograph showed multiple nodular opacities on left upper lobe. He was treated with anti-tuberculosis drugs in our hospital. Six months after the therapy, he began to complain of dry cough and shortness of breath on exertion. Chest radiograph showed reticular opacities in both upper and middle lung fields. Multiple thick- and thin-walled bizarre-shaped cysts and ill-defined centrilobular opacities were seen mainly in upper and mid-lungs on high-resolution computed tomography (HRCT). He was diagnosed with pulmonary Langerhans-cell histiocytosis (PLCH) by transbronchial lung biopsy specimens from right upper lobe. HRCT findings before the treatment of tuberculosis were retrospectively examined. Several small thin-walled centrilobular cysts were scattered in the right upper and middle lung fields without apparent nodular lesions. We must consider the possibility of PLCH when these findings are detected on HRCT.     

Changes in HRCT findings in patients with respiratory bronchiolitis-associated interstitial lung disease after smoking cessation.

High-resolution computed tomography (HRCT) findings in patients with respiratory bronchiolitis-associated interstitial lung disease (RB-ILD) are varied and nonspecific. There is no known report of changes in HRCT findings and respiratory function test results for RB-ILD patients following the cessation of smoking. Five patients with RB-ILD, confirmed by surgical lung biopsy, were retrospectively studied. Each stopped cigarette smoking and did not receive corticosteroid therapy after diagnosis. The clinical symptoms, respiratory function test results and HRCT findings obtained at the final observation were compared with those from the time of diagnosis. Ground-glass opacity and centrilobular nodules corresponding to pathological respiratory bronchiolitis, as well as intralobular fine linear-reticular opacity corresponding to fibrosis involving the subpleural alveolar septa, showed computed tomography-pathological correlations. Both clinical symptoms and the diffusing capacity of the lungs for carbon monoxide improved significantly following smoking cessation, as did ground-glass opacity and centrilobular nodules seen during the initial HRCT examination. Centrilobular nodules and ground-glass opacity, which are the main features of high-resolution computed tomography of respiratory bronchiolitis-associated interstitial lung disease patients and represent pathological respiratory bronchiolitis, can be improved by smoking cessation. The diffusing capacity of the lung for carbon monoxide in respiratory function tests can be also improved.     

The overlap between respiratory bronchiolitis and desquamative interstitial pneumonia in pulmonary Langerhans cell histiocytosis: high-resolution CT, histologic, and functional correlations

OBJECTIVES: To characterize the histologic, high-resolution CT (HRCT), and functional correlates of respiratory bronchiolitis (RB)/desquamative interstitial pneumonia (DIP)-like changes in biopsy specimens of adults with pulmonary Langerhans cell histiocytosis (PLCH). METHODS: We retrospectively identified 14 adult patients with histologically proven PLCH (all smokers) in whom both biopsy specimens and HRCT were available for review. The presence and extent of RB/DIP-like changes on lung biopsy specimens of patients with PLCH were correlated with total pack-years of smoking, lung function variables, and the presence of ground-glass attenuation on HRCT. RESULTS: Varying degrees of RB/DIP-like changes were identified in all biopsy specimens. The extent of involvement with RB/DIP-like changes correlated with the cumulative exposure to cigarettes smoked at the time of biopsy (r = 0.61, p = 0.03). Ground-glass attenuation was detected in three HRCTs, two of which had extensive RB/DIP-like changes (> or = 70% involvement) on histology, suggesting that substantial smoking-induced RB/DIP-like changes may be the cause of ground-glass attenuation on the HRCT of patients with PLCH. No correlation was found between the extent of RB/DIP-like changes and total lung capacity, FEV(1), or diffusion capacity of the lung for carbon monoxide. CONCLUSIONS: RB/DIP-like changes are exceedingly common in PLCH, may be sufficiently severe to cause the appearance of ground-glass attenuation on HRCT, and correlate with the cumulative exposure to cigarettes smoked. This study provides additional evidence that PLCH, RB, and DIP form a spectrum of interstitial patterns of lung injury to cigarette smoke.     

A case of pulmonary Langerhans cell histiocytosis discovered by CT mass screening and followed by bronchoalveolar lavage]

A 42-year-old woman who had a cigarette index of 420 had many cavitary lesions predominantly in the upper areas of both lungs. The lesions were detected on a health examination using CT. Analysis with bronchofiberscopy showed increased CD1a positive cells in bronchoalveolar fluid (BALF). Histological examination by video-associated lung biopsy demonstrated that both S-100 and vimentin-positive cells were present in the peribronchial fibrotic lesions. From these data, this disease was diagnosed as pulmonary Langerhans cell histiocytosis (PLCH). Three months after the cessation of smoking, the cavity lesions disappeared. As the image findings improved, CD1a positive cells in BALF decreased (4.9% --> 1.8%) and the CD4/CD8 ratio in BALF increased (1.66 --> 6.16). So far, there is no report describing the time course of both CD1a positive cells and CD4/CD8 ratio in BALF after cessation of smoking in PLCH. These findings attract our interest on the PLCH.     

Pulmonary Langerhans Cell Histiocytosis: Case Series and Literature Review

Pulmonary Langerhans cell histiocytosis (PLCH) is a rare disease with insidious onset and nonspecific manifestations. The objective of this article was to characterize the clinical manifestations and features of PLCH by retrospectively analyzing clinical data of patients with PLCH in addition to simultaneous review of literature.A retrospective analysis was conducted on clinical data of patients with PLCH (n = 7), whose conditions were diagnosed by biopsy from pulmonary tissue (n = 6) or enlarged lymph nodes in the neck (n = 1) and confirmed by PLCH typical radiological features on computed tomography (CT) scan, between January 2001 and September 2012 at the Shanghai Pulmonary Hospital, Tongji University School of Medicine, Shanghai, China. The review of published reports was made to further emphasize the clinical manifestation and radiological features of PLCH.Long history of cigarette smoking was found in 6 patients. Two patients had recurrent pneumothorax and the other 2 had pulmonary arterial hypertension (World Health Organization group 5 pulmonary hypertension), diagnosed through ultrasonic cardiogram. The nodular shadows were revealed by chest CT scan in 5 patients, cystic shadows in 5 patients, and reticular shadows in 2 patients, as major manifestations, respectively; most of the lesions were located in the middle or upper segments of the lung. The obvious shrank of lesion was found in 1 patient after completely quitting smoking.The pathogenesis of PLCH might be closely associated with smoking. The cystic or nodular lesion was the typical radiological features. Further prospective studies with large sample size are required to further validate the study results and understand the clinical characteristics of PLCH to avoid misdiagnosis.     

Pulmonary Langerhans cell histiocytosis: emerging concepts in pathobiology,radiology, and clinical evolution of disease

Pulmonary Langerhans cell histiocytosis (PLCH) is an uncommon disorder of adult smokers associated with a significant morbidity. Arising from the aberrant accumulation of Langerhans and other immune cells, PLCH tends to cause a relatively isolated pulmonary involvement as compared to other forms of Langerhans cell (LC) and histiocytic disorders. Increased knowledge of cytokine triggers, dendritic cell trafficking, and clonality of LC populations in PLCH have resulted in an improved understanding of the pathobiology of PLCH. High-resolution CT (HRCT) of the chest has led to better appreciation of nodular and cystic radiographic abnormalities characteristic of the disease. Correlation of HRCT abnormalities with lung pathologic changes has led to an improved comprehension of clinical evolution of PLCH. Current clinical predictors for PLCH outcomes remain poor, although long-term follow-up and radiologic monitoring may help to define disease progression. This review discusses advances in PLCH emphasizing the etiopathologic bases of the disease and currently available radiologic modalities for monitoring disease progression.     

A comparative study of computed tomographic techniques for the detection of emphysema in middle-aged and older patient populations]

Helical-scan computed tomography (CT) is now widely utilized as a mass screening procedure for lung cancer. By adding 3 slices of high-resolution CT (HRCT) to the standard screening procedure, we were able to compare the efficacy of helical-scan CT and HRCT in detecting pulmonary emphysema. Additionally, the prevalence of emphysema detected by HRCT was examined as a function of patient age and smoking history. The subjects (106 men and 28 women) were all community-based middle-aged and older volunteers who participated in a mass lung cancer screening program. Based on visual assessments of the CT films, emphysema was detected in 29 subjects (22%) by HRCT, but in only 4 (3%) by helical-scan CT. Although the prevalence of emphysema was higher among subjects with a higher smoking index, no correlations with age were observed. We concluded that the efficacy of helical scan CT in detecting pulmonary emphysema can be significantly improved with the inclusion of 3 slices of HRCT, and confirmed that cigarette smoking is linked to the development of pulmonary emphysema.     

Respiratory bronchiolitis-associated interstitial lung disease

Respiratory bronchiolitis-associated interstitial lung disease (RBILD) can be viewed as an exaggerated respiratory bronchiolitic response to cigarette smoke. The histologic, high-resolution computed tomographic (HRCT) and bronchoalveolar lavage (BAL) features of RBILD overlap substantially with those of respiratory bronchiolitis, with the diagnosis of RBILD being based upon the severity of disease, as judged by symptoms, clinical signs, the severity of lung function impairment, and the extent of abnormalities on HRCT. Typical histologic appearances consist of an accumulation of pigmented macrophages within respiratory bronchioles, associated with peribronchial chronic inflammatory cell infiltration and, variably, peribronchial fibrotic alveolar septal thickening. Characteristic HRCT findings include poorly defined centrilobular micronodules, patchy limited ground-glass attenuation, bronchial wall thickening, and areas of regional hypoattenuation. The ventilatory defect is often mixed but is usually predominantly restrictive. The diagnosis of RBILD is often made on clinical and HRCT criteria, with BAL findings providing useful diagnostic support, but a thoracoscopic biopsy continues to be required when other features are atypical. RBILD may regress with discontinuation of smoking but often persists with no functional improvement despite smoking cessation and treatment. Nonetheless, the course tends to be benign, without inexorable deterioration. This article outlines the rationale for viewing RBILD and desquamative interstitial pneumonia as separate entities, rather than two ends of the same disease spectrum (based upon overlapping histologic and HRCT features).