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。     

结缔组织病相关的肺动脉高压研究进展

肺动脉高压是结缔组织病（CTD）的严重并发症之一。结缔组织病中最常受累的是系统性硬化症（SSc）,而混合性结缔组织病（MCTD）、系统性红斑狼疮（SLE）及炎性肌病包括多肌炎和皮肌炎也常被累及。结缔组织病相关的肺动脉高压引起的原因不仅是肺动脉的病变,慢性血栓的形成,自身免疫反应和肺间质病变也是引起肺动脉高压的原因之一。由于其症状出现晚且不典型,预后甚差,死亡率高,因此对结缔组织病相关的肺动脉高压早期诊断,及时合理的治疗十分重要。     

系统性硬化病合并肺动脉高压临床分析

目的：探讨系统性硬化病（SSc）合并肺动脉高压（PHT）的临床特点。方法：回顾性研究18例SSc合并PHT患者的临床特点，实验室以及影像学检查，并与58例未合并PHT的SSc患者进行比较。结果：与表合并PHT患者血清中IgA、IgG以及C反应蛋白（CRP）水平间差别均有显著性意义（P〈0．05）。肢端病变、肌炎、肺纤维化，ECG异常、肾脏受累的发生率及抗RNP阳性率高于非PHT患者（P〈0．05）。结论上述指标有助于发现合并PHT的可能性，定期对SSc患者行超声心动图检查对筛查PHT非常必要。PHT是SSc的一个严重并发症，多伴有其他脏器损害，预后不良，应尽早采取综合治疗，尤其是针对原发病和诱因的强化治疗，糖皮质激素、免疫抑制剂、血管扩张剂和抗凝剂是治疗PHT的基本药物，病情顽固患者用前列环素制剂可改善预后。     

定量CT在评估类风湿性关节炎相关间质性肺病中的应用

目的:探究胸部CT定量分析对于类风湿性关节炎相关间质性肺病（RA-ILD）患者肺间质改变早期诊断及病情评估的价值。方法:收集临床确诊RA患者105例,同时收集胸部CT无间质改变的非结缔组织病患者对照组80例。测量两组研究对象肺总体积（TLV）,利用定量CT肺部密度直方图计算CT值为-200~-700 HU的肺体积占全肺体积百分比（LAA-200~-700%）、CT值小于-950 HU的肺体积占全肺体积百分比（LAA-950%）、主动脉直径（AD）、肺动脉干直径（PAD）、主肺动脉干比值（AD/PAD）、左肺下叶直径2 mm支气管周围血管数量及面积（视区范围80 mm2）。比较RA患者组与对照组的测量值差异。结果:RA患者组与对照组相比,TLV、LAA-200~-700%、PAD、左肺下叶直径为2 mm的支气管视区范围为80 mm2时肺血管数量及面积均存在差异,其中RA患者组TLV为（4 047.60±1 160.11） mL,小于对照组（4 507.30±1 207.50） mL;LAA-200~-700%为14.42%±8.62%,大于对照组10.40%±3.87%,PAD为（26.39±3.59） mm,大于对照组（25.27±2.57） mm;RA组左肺下叶直径为2 mm支气管层面视区范围为80 mm2时肺血管数量为13.3±6.28,面积为（105.48±59.07） mm2;对照组血管数量为17.06±4.70,血管面积为（164.88±46.02） mm2,差异有统计学意义（P<0.001）。结论:RA-ILD患者肺部与对照组相比有差异,定量CT能有效识别评估RA-ILD早期肺血管改变。     

肺结核患者血浆内皮素与痰菌结果及病变范围的临床观察

目的探讨肺结核患者血浆内皮素变化与痰菌结果及病变范围的关系及临床意义。方法继发型肺结核患者68例,对照组30例,入院后次日抽取空腹静脉血液4 ml待测ET-1。痰涂片阴性（菌阴）38例,痰涂片阳性（菌阳）30例。按胸部正位片上病变范围分为≤2肺野26例,病变范围〉2肺野42例。结果结核组血浆ET-1水平显著高于对照组（P〈0.01）,菌阳组血浆ET-1水平显著高于菌阴组（P〈0.01）,病变范围〉2肺野与≤2肺野比较血浆ET-1水平显著增高。痰菌阳性、肺部病变范围越大,病变程度越重,则血浆ET-1水平越高。结论肺结核患者血浆ET-1水平与痰菌结果及病变范围相关。     

新风胶囊对佐剂性关节炎大鼠肺部HRCT及血清细胞因子的影响

目的：观察新风胶囊对佐剂性关节炎（Adjuvant arthritis,AA）大鼠肺部高分辨率CT（High resolution ratio CT, HRCT）改变及血清细胞因子的影响.方法：将60只大鼠随机分为正常对照组、模型对照组、甲氨喋呤（Methotrexate,MTX）组、雷公藤多苷片（Tripterygium wilfordii polycoride tablet,TPT）组和新风胶囊（Xinfeng capsule,XFC）组,每组12只,分别向除正常对照组以外的其余动物右后足跖皮内注射弗氏完全佐剂0.1 ml致炎,致炎后第19天开始给药.正常对照组及模型对照组均给予生理盐水,其余3组分别给予MTX、TPT、XFC.给药1个月后,观察各组大鼠肺部HRCT及血清细胞因子的变化.结果：HRCT表现为模型对照组大鼠肺轮廓欠清晰,肺部血管影扩张,边缘模糊且不规则,两肺有索条状密度增高影,邻近胸膜轻度肥厚;甲氨喋呤组大鼠肺部可见沿着肺纹理分布的斑片状密度增高影,肺血管纹理模糊,边缘不清楚;雷公藤多苷片组大鼠两肺可见多发性结节状及斑片状密度增高影,境界欠清晰;新风胶囊组大鼠肺轮廓清晰,肺纹理分布均匀,部分肺组织显示斑点状密度增高影.与正常对照组比较,模型对照组TNF-α显著升高（P＜0.05）;与模型对照组比较,新风胶囊组TNF-α显著降低（P＜0.01）,而与其余2治疗组无显著差异（P＞0.05）;与正常对照组比较,模型对照组IL-10显著降低（P＜0.01）;与模型对照组比较,新风胶囊组IL-10显著升高（P＜0.01）,而与其余2治疗组无显著差异（P＞0.05）.结论：新风胶囊能下调致炎因子TNF-α、上调抗炎因子IL-10、抑制致炎效应、增强抗炎效应,在降低AA大鼠足跖肿胀度的同时,也能改善肺部影像学改变.     

多层螺旋CT在诊断肺间质性病变的观察及应用价值

目的探讨多层螺旋CT在鉴别诊断肺间质性病变的临床价值；方法选择26例肺间质性病变患者的临床资料，分析 X线胸片、常规 CT和 HRCT检测结果，并与病理与随访结果比较；结果 HRCT检测敏感度95.45%（21/22），特异性75%（3/4），准确率92.31%（24/26），均明显高出 X线片和常规 CT（P<0.01或P<0.05）；结论 HRCT能够较准确地检测肺间质性病变的细微变化，确定病变性质和范围，具有积极的临床意义。     

肺部超声对重症肺部感染患者肺通气的评估价值

目的:探讨肺部超声评价重症肺部感染患者通气情况的应用价值。方法:选取88例重症肺部感染患者,采用半定量方法对肺部超声征象进行评分,以CT检查结果为金标准,分析肺部超声评分与患者肺通气的关系;同时分析存活和死亡患者临床资料、肺部超声评分的差异,以及肺部超声评分预测患者死亡的价值。结果:88例患者全肺超声评分平均为（18.50±2.12）分,全肺CT值平均为（-620.50±88.13） HU,不通气/低通气肺组织比例平均为（10.41±3.35）%,正常通气肺组织比例平均为（71.54±6.69）%,过度通气肺组织比例平均为（17.65±4.11）%;患者肺部超声评分与全肺CT值、不通气/低通气肺组织比例呈正相关（r=0.775、0.648, P<0.05）,与正常通气肺组织比例、过度通气肺组织比例无明显相关性（r=-0.170、0.046, P>0.05）;死亡组患者年龄、糖尿病比例、APACHEⅡ评分、肺泡-动脉氧分压差、机械通气治疗和肺部超声评分分别为（59.28±8.12）岁、44.83%、（22.19±2.40）分、（344.40±82.29） mmHg、72.41%和（20.20±1.72）分,明显高于存活组（P<0.05）,而氧合指数为（104.42±21.18）,明显低于存活组（P<0.05）;Logistic回归分析结果显示:年龄、APACHEⅡ、肺部超声评分是重症肺部感染患者死亡的影响因素（OR=1.758、2.841、2.440, P<0.05）;肺部超声评分预测重症肺部感染患者死亡的ROC曲线下面积为0.901（95%CI:0.836~0.966）,截断值为20分,灵敏性和特异性分别为82.80%和84.70%。结论:肺部超声可以作为重症肺部感染患者肺通气的评估指标,同时其在预测患者预后方面有一定应用价值。     

结核感染T细胞斑点试验与肺部CT表现的相关性

目的:探讨活动性肺结核患者结核感染T细胞斑点试验（T-SPOT.TB）数值测定与高分辨CT（HRCT）征象评分的相 关性。方法:回顾分析52例肺结核患者,对其外周血液标本进行T-SPOT.TB数值测定,并根据其HRCT的7种典型征象分 布及范围行对上述患者的肺部CT表现进行评分,利用结核感染T-SPOT.TB数值与HRCT征象评分进行散点图绘制,统计 学软件进行相关性分析,讨论两者有无相关性。结果:52例患者肺部HRCT评分分值分布在2~52。微结节分值最大,总 分为254,平均4.88;最小为空洞,总分10,平均0.19,分值整体上成正态分布;52 例患者T-SOPT.TB所测值A抗原孔阴性 （测得值为0~5）17例,阳性（测得值>5）35例,B孔抗原阴性（测得值为0~5）14例,阳性（测得值>5）38例,T-SPOT.TB表现为 阴性所对应的HRCT征象评分均值与阳性所对应均值对比无统计学差异（不论A孔或者B孔）;从散点图看T-SOPT.TB所 测A抗原孔及B抗原孔值与HRCT征象评分值之间存在一定相关性,利用统计学计算相关系数分别为0.27（P<0.05）和 0.17（P<0.05）,提示T-SOPT.TB测定值与HRCT征象评分为正相关性。结论:T-SOPT.TB与肺部CT所示肺结核病变范围 大小及程度有一定相关性。     

射野角度优化方法在全段食管癌IMRT计划设计中的应用

目的:分析射野角度优化（BAO）布野、均分布野和人工布野3种5野全段食管癌IMRT计划设计方法的剂量学差异，为全段食管癌IMRT计划设计提供临床参考。方法:选取15例行IMRT治疗的全段食管癌患者，分别设计3种5野IMRT（5f-IMRT）计划:基于BAO方法布野计划组（A组）、均分布野计划组（B组）以及人工布野计划组（C组）。用靶区覆盖率、适形度指数（CI）和均匀性指数（HI）以及各危及器官的剂量学参数来比较3组计划方法的剂量学差异，统计分析用配对t检验方法。结果:3组计划的靶区剂量覆盖均能满足临床要求。对于危及器官，A组计划的双肺Dmean和心脏平均值明显低于其他两组计划，差异有统计学意义（P<0.05）。相对于C组计划，A组和B组计划中双肺的V20 Gy和心脏V30 Gy均明显降低（P<0.05），其中A vs C、B vs C的双肺V20 Gy和心脏V30 Gy分别为［（29.15±2.75）% vs （30.03±2.55）%, P=0.005］、［（29.08±2.40）% vs （30.03±2.55）%, P=0.005］和［（45.37±4.59）% vs （48.24±6.45）%, P=0.010］、［（45.79±4.60）% vs （48.24±6.45）%, P=0.040］。最后，对3组计划的剂量学参数进行综合评分，其中A组计划中的靶区PTVx综合评分、危及器官综合评分和计划综合评分得分均为最高，其中计划综合评分CSFTotal为7。C组计划综合评分最差，CSFTotal为-4。结论:相比于人工布野方法与均分布野方法设计的全段食管癌5f-IMRT计划，BAO计划具有明显的剂量学优势，建议在全段食管癌的计划设计中推广应用。     

中国系统性硬化症患者硬皮病相关自身抗体的检测及其临床意义

目的 检测中国系统性硬化症(SSc)患者血清中硬皮病相关自身抗体-抗Sol-70抗体、抗着丝点抗体(ACA)和抗RNA多聚酶Ⅲ抗体(ARA),分析其与各种临床表现之间的关系.方法 序贯纳人入选欧洲抗风湿病联盟硬皮病实验研究组(EULAR Scleroderma Trial and Research Group,EUSTAR)的135例巾国SSc患者,分别用线性免疫印迹法、免疫双扩散法和间接免疫荧光法检测ARA、抗Scl-70抗体、ACA在患者血清中表达水平,并进一步分析自身抗体与患者各种临床表现之间的相关性.结果 在135例SSc患者中抗Sel-70、ACA、ARA的阳性率分别为49 6%、13 3%和8.9%.抗Scl-70抗体阳性组患者的病程显著短于阴性组[(71±59)个月vs(90±103)个月,P=0 041],肺问质病变的患病率亦显著高于阴性组(P=0.031),但阳性组肺动脉高压的患病率显著低于阴性组(P=0 042),修订的Rodnan皮肤硬化评分(P=0 008)、面颈部皮肤硬化(P=0.002)、肘/膝关节远端皮肤硬化(P=0.004)以及指端凹陷性瘢痕/指垫消失的发生率(P=0.01)均显著高于阴性组;ACA附性组患者的病程长于阴性组,差异具有统计学意义[(90±107)个月vs(69±64)个月,P=0.036],肺间质病变的患病率显著低于阴性组(P=0 045),IgM水平亦显著低于阴性组(P=0 045);ARA阳性组和阴性组患者的病程等各项临床指标差异均无统计学意义,但阳性组血清肌酐和尿素氨水平显著高于阴性组(P＜0.001).ACA和ARA患者各项皮肤硬化指标在阳性组和阴性组差异均尤统计学意义.结论 硬皮病特异相关的自身抗体与不同的临床表现紧密相关,检测此类抗体可能有助于SSc的诊断、脏器受累和预后评估.这些自身抗体在中国SSc患者的临床相关性可能不同于其他地区的SSc患者.

Abstract：

Objective To detect the expression of scleroderma-related autoantibodies, such as anti-Scl-70, anli-centromere antibody ( ACA)and anti-RNA polymerase Ⅲ ( ARA) , and their relationship with clinical features in Chinese systemic sclerosis (SSc) patients. Methods One hundred and thirty-five Chinese SSc patients from the clinical database of the Scleroderma Trials and Research Group proposed by European League Against Rheumatism's Scheroderma Trial and Research Group( EUSTAR) were consecutively enrolled. The expression of ARA, anti-Scl-70 and ACA were detected through linear immunoblotting, double immunodiffusion and indirect irnmunofluorescence, respectively. The relevance between the existing of autoantibodies and clinical manifestations was analyzed statistically. Results Among the 135 Chinese SSc patients, the prevalence of anti-Scl-70, ACA, ARA were 49. 6% , 13.3 % and 8.9% respectively. Patients with anti-Scl-70 antibody had significantly shorter disease course [(71 ±59) month vs (90 ± 103) month, P = 0.041] , higher proportion of interstitial lung disease ( P = 0. 031) but lower of pulmonary arterial hypertension (P =0.042). Modified Rodnan's skin score (P=0.008) and prevalence of facial and cervical cutaneous sclerosis (P = 0. 002) , distal (to elbow/knee ) cutaneous sclerosis ( P = 0. 004 ) and digital pitting scarring/disappear of digital pad were all significantly higher in anti-Scl-70 positive group. Patients with AC A had longer disease course ( P = 0. 036) , lower IgM level ( P = 0. 045) and were less prevalent of interstitial lung disease ( P =0. 045). Patients with ARA had higher serum creatinine and urea nitrogen level ( P ＜ 0.001) although otherwise features had unremarkable differences. Conclusion Scleroderma-related autoantibodies have relevance with different clinical manifestation and detection of these autoantibodies may be helpful to the diagnosis of SSc, organ involvement evaluation and predicting outcomes. The clinical relevances of autoantibodies in Chinese SSc patients may differ from other areas or races.     

Anti-carbonic Anhydrase II Antibodies in Systemic Sclerosis: Association with Lung Involvement

Carbonic anhydrase II (CAII) is expressed on alveolar epithelium and participates to CO 2 elimination, fluid secretion and post-capillary pH regulation. CAII is overexpressed in animal models of lung fibrosis in sites of epithelial injury. Autoantibodies directed against CAII (anti-CAII) have been described in sera from patients affected by systemic sclerosis (SSc), but no study focused on their clinical associations in this disease. The aim of this study was to assess the presence of anti-CAII in sera of SSc patients and to investigate their association with lung involvement. We performed ELISA to detect anti-CAII in 34 SSc patients who underwent pulmonary function tests (PFT) and Doppler echocardiography. We found increased prevalence and significantly elevated serum levels of anti-CAII in SSc patients affected by restrictive lung disease (RLD) compared to SSc patients without lung involvement and healthy controls. These findings suggest both a possible pathogenic role of anti-CAII in the development of lung damage and a potential clinical utility as serological marker of pulmonary involvement in SSc patients.     

Scleroderma Lung Disease

Pulmonary involvement is second in frequency only to esophageal involvement as a visceral complication of systemic sclerosis (SSc) and has surpassed renal involvement as the most common cause of death. Interstitial lung disease and pulmonary vascular disease, particularly pulmonary arterial hypertension, are the most commonly encountered types of lung involvement. Chronic aspiration, airway disease, neuromuscular weakness, extrinsic pulmonary restrictive pathology, pleural effusions, pneumothorax, and lung cancer cause clinically significant disease and occur commonly enough to be routinely considered in the assessment of the SSc patient with respiratory symptoms. Affected patients have a significantly worse prognosis than patients with SSc who are free of pulmonary involvement.     

儿童先天性体动脉-肺动脉瘘4例并文献复习

目的总结临床表现为大量咯血的先天性体动脉-肺动脉瘘患儿的临床特点及诊治经验。方法回顾性收集首都医科大学附属北京儿童医院2007年3月至2008年2月诊断为先天性体动脉-肺动脉瘘4例患儿的临床资料,总结其临床表现、胸部X线片、胸部64排CT增强扫描三维容积再现（3D-VR）、数字减影血管造影（DSA）、治疗及随访情况。结果男1例,女3例,最大发病年龄为11岁,最小发病年龄为2个月,主要表现为咯血。1例有杵状指,3例未见特异性体征。胸部X线片检查：3例未见异常,1例示肺间实质浸润。胸部64排CT增强扫描3D-VR检查：1例提示支气管动脉迂曲,但未见异常交通;1例导管栓塞治疗（TCE）后示右侧支气管动脉扩张迂曲,并与肺动脉相通可能性大,考虑支气管动脉-肺动脉瘘;1例提示支气管动脉-肺动脉瘘;1例未见异常。3例DSA提示为支气管动脉-肺动脉瘘,病变部位均位于右下肺,并行TCE。随访至2009年2～5月,1例复发,表现为痰中带血,胸部64排CT增强扫描未见异常,余3例未复发。3例怀疑为遗传性出血性毛细血管扩张症（HHT）,1例考虑为HHT高度危险者。结论体动脉-肺动脉瘘可造成大量咯血。胸部X线片一般无特异性表现,胸部64排CT增强扫描3D-VR可显示病变部位,明确诊断需行DSA。可采用TCE治疗。TCE远期效果应进行长期随访予以明确。     

Serum 25-OH vitamin D concentrations are linked with various clinical aspects in patients with systemic sclerosis: a retrospective cohort study and review of the literature

Low vitamin D serum concentrations have been reported in several autoimmune conditions. The study's aim was to explore such a relationship in a large multinational population of patients with systemic sclerosis (SSc) and to pursue possible clinical and laboratory correlates with vitamin D concentrations. 327 sera samples of European patients with SSc and 141 samples of compatible healthy controls were studied for vitamin D concentrations using the commercial kit LIAISON 25-OH vitamin D assay (Diasorin). Additionally, clinical parameters including the Rodnan skin score, diffusing lung capacity for carbon monoxide (DLCO), systolic pulmonary artery pressure (sPAP), forced vital capacity (FVC), and nailfold video capillaroscopic, erythrocyte sedimentation rate (ESR), anti-nuclear antibodies (ANA and scl70), rheumatoid factor (RF) were investigated. Vitamin D serum concentration was 13.5 ± 9.0 ng/ml (mean ± standard deviation) in patients with SSc compared to 21.6 ± 9.7 ng/ml in a control group (p<0.001). A negative correlation between patients' age and vitamin D concentration (r = -0.2, p<0.05, n = 96) was observed. An inverse relationship was found between skin involvement and vitamin D serum concentrations; Patients with a Rodnan skin score of 10 or lower (n = 11) had a mean vitamin D concentration of 17.7 ± 10.4 ng/ml compared to patients with a score above 10 (n = 28) 8 ± 10.1 ng/ml (p=0.02, by the Mann-Whitney test). In conclusion, Patients with SSc have significantly lower serum vitamin D concentrations compared to healthy controls; moreover fibrosis of the cutaneous tissue is inversely related to the vitamin D concentration.     

Mean Platelet Volume Seems To Be a Valuable Marker in Patients with Systemic Sclerosis

The predictors for the development of cardiovascular diseases and peripheral arterial diseases in patients with systemic sclerosis (SSc) were not clearly established, and there is no specific study conducted to investigate the mean platelet volume (MPV) levels in SSc patients. Therefore, this study evaluates the MPV levels in SSc and possible relationship between SSc, its clinical features and activity/severity scores, and MPV. In total, 76 SSc patients (67 women and 9 men, mean age 50.44?±?13.21 years) diagnosed according to the classification criteria of the American College of Rheumatology and 45 healthy volunteers were enrolled into study. Data relating to anamnesis, physical examination, MPV, erythrocyte sedimentation rate, C-reactive protein levels, electrocardiography, echocardiography, high-resolution computerized tomography findings, complaints, and treatment processes were recorded into the database. Of the total cases, 17 had (22.3 %) cardiac involvement, 45 had gastrointestinal involvement (59.2 %), 47 had (61.8 %) lung involvement, 31 (32 %) had finger flexion deformity, and 27 (35.5 %) had digital ulcers at the fingertips. The mean MPV levels of SSc patients were significantly higher than those of the control group (p?=?0.008). The mean MPV levels of SSc patients with cardiac involvement, digital ulcers, and gangrene presence were significantly high, and lower in Ilomedin-receiving patients than in the Ilomedin naives (p?<?0.05). A negative relationship was discovered between the mean MPV levels, Valentini score, and Disease Severity Index of the patients with systemic sclerosis (p?=?0.006, r?=??0.310; p?=?0.047, r?=??0.229). MPV levels were significantly elevated in SSc patients and they were negatively correlated with disease activity scores. Increased MPV levels would be a predictive marker in the diagnosis of macrovascular and microvascular disease involvement in SSc patients.     

Distinctive autoantibody profile in Mexican Mestizo systemic sclerosis patients

Systemic sclerosis (SSc) shows variable clinical expression among different ethnic groups. Herein, we describe the clinical features, prevalence of organ involvement, and autoantibody profile in Mexican Mestizo SSc patients and we compare them with patients from other ethnic groups.We included 139 SSc patients. They underwent clinical evaluation and were tested for antinuclear antibodies (ANA), anticentromere antibodies (ACA), anti-topoisomerase I, anti-RNA polymerase III, anti-U1 RNP, anti-U3 RNP, anti-U11/U12 RNP, anti-Th/To, anti-PM-Scl, anti-Ku, antinucleosome, anti-double-stranded DNA (dsDNA), anti-Sm, anti-SSA, and anti-SSB antibodies. Female predominance (93.5%) was noted; 56.8% of patients had limited cutaneous SSc; 91% had peripheral vascular involvement; 70% had joint involvement; 27% had musculoskeletal damage; 66% had gastrointestinal involvement; 41% had interstitial lung disease; 32% had pulmonary arterial hypertension (PAH); 11% had cardiac involvement; and in 1.4% renal involvement was observed. Our patients showed lower frequency of renal crisis and higher frequency of PAH than patients from other ethnic groups; also they showed higher frequency of ACA than Japanese and African American patients, higher frequency of anti-topoisomerase I than Caucasian and African American patients, higher frequency of anti-PM-Scl and anti-Ku and lower frequency of anti-RNA Pol III than the other ethnic groups. High frequencies of antinucleosome (41%) and anti-dsDNA (63%) were identified. SSc-specific autoantibody frequencies are different in our patients and in those from other ethnic groups; associations of autoantibodies with clinical manifestations are confirmed in our patients. Ethnicity and the interaction of gene and environmental factors may influence the clinical picture and autoantibody profile in SSc patients.     

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。     

bFGF、MMP-2在肺血减少型先天性心脏病幼猪动物模型远端肺血管发育中的作用

目的:采用球囊扩张人工房间隔造口+肺动脉环缩术建立肺血减少型先天性心脏病幼猪动物模型,探讨肺血减少时远端肺血管形态学变化的病理生理机制.方法:将出生1～2个月的幼猪20头随机分为3组.对照组(C组,n=6):右胸小切口制成一过性肺血减少;轻度-中度肺动脉狭窄组(T1组,n=7):经右心房表面送入球囊扩张器,行人工房间隔造口+肺动脉Banding环缩术,控制收缩期肺动脉环缩处压差(Trans-PABP)为20～30 mmHg;重度肺动脉狭窄组(T2组,n=7):术中Trans-PABP≥30～50 mmHg.三组于术后2个月取右肺中叶外侧段1.0 cm×0.8 cm×0.8 cm大小的肺组织,光学显微镜下观察远端肺细小动脉形态学改变,并采用双抗体夹心酶联免疫吸附法(ELlSA)检测血清碱性成纤维细胞生长因子(bFGF)和基质金属蛋白酶2(MMP-2)的含量.结果:T1、T2组行房间隔造口+肺动脉环缩术均获成功.术后2个月,T1、T2两组肺细小动脉内径均大于C组(P＜0.05),肺细小动脉数目(NAPSC)均少于C组(P＜0.05).血清bFGF 、MMP-2含量,T1、T2两组较C组升高(P＜0.05),而C组手术前后没有明显变化(P＞0.05).结论:肺血减少型先天性心脏病幼猪,肺组织细胞外基质发生构型重建,肺细小血管发育不良或退化,其发生基质重塑的基础是肺血减少时引起一系列酶和细胞因子等成分的变化.     

Anti-carbonic anhydrase II antibodies in systemic sclerosis: association with lung involvement

Carbonic anhydrase II (CAII) is expressed on alveolar epithelium and participates to CO2 elimination, fluid secretion and post-capillary pH regulation. CAII is overexpressed in animal models of lung fibrosis in sites of epithelial injury. Autoantibodies directed against CAII (anti-CAII) have been described in sera from patients affected by systemic sclerosis (SSc), but no study focused on their clinical associations in this disease. The aim of this study was to assess the presence of anti-CAII in sera of SSc patients and to investigate their association with lung involvement. We performed ELISA to detect anti-CAII in 34 SSc patients who underwent pulmonary function tests (PFT) and Doppler echocardiography. We found increased prevalence and significantly elevated serum levels of anti-CAII in SSc patients affected by restrictive lung disease (RLD) compared to SSc patients without lung involvement and healthy controls. These findings suggest both a possible pathogenic role of anti-CAII in the development of lung damage and a potential clinical utility as serological marker of pulmonary involvement in SSc patients.