系统性硬化症肺部高分辨率CT影像学表现分析

目的探讨系统性硬化症(SSc)肺部高分辨率CT(HRCT)影像学特征。方法回顾性分析2013-02~2017-04该院临床确诊15例SSc肺部HRCT影像学表现。结果 15例SSc患者肺部正常4例,表现以两肺外围分布为主的间质性纤维化11例。HRCT主要表现支气管血管束扭曲变形、小叶间隔增厚,可见条索状影、胸膜下线及弧形线9例,呈网格状改变7例。沿支气管血管束及小叶间隔分布小结节状影,胸膜下见小结节影10例。单纯以磨玻璃样改变5例,轻度胸膜增厚或少量胸腔积液9例。小片状实变影及蜂窝肺4例。结论 HRCT显示SSc的间质性细微病变,在SSc的诊断和随访治疗中具有重要价值。     

结缔组织病肺间质病变的临床特点及治疗效果分析

目的探讨不同结缔组织病肺间质病变（CTD-ILD）的临床特点和治疗效果。方法分析82例CTD-ILD患者的临床特征及胸部高分辨CT（HRCT）特点并观察治疗后HRCT的变化。结果（1）SSc—ILD的发生率最高，为70．0％。其次为pSS—ILD、PM／DM-ILD、RA—ILD、SLE—ILD，系统性红斑狼疮继发的ILD（SLE—ILD）发病年龄（26．2±7．8）较其他CTD-ILD早（P〈0．05）。RA—ILD、SSc—ILD病程较长，pSS—ILD、SLE一[LD和PM—ILl）病程较短。RA．ILD出现活动后气促少见（P〈0．05）。pSS—ILD出现呼吸道症状（40％）和体征（50％）最多见。85．7％SSc-ILD出现雷诺现象，支持雷诺现象与ILD相关。（2）SLE—ILD以磨玻璃影多见，丽蜂窝状改变较少；pSS-ILD以蜂窝状改变和纵隔淋巴结肿大相对较多。（3）大部分患者治疗后肺部间质病变吸收好转（56．7％），其中88．2％SLE—ILD吸收好转，28．4％患者肺部病变处于静止，14．9％患者间质病变较前进展。结论CTD—ILD患者一旦确诊，应尽早用激素联合免疫抑制剂治疗。     

几种弥漫性结缔组织病肺间质病变的影像分析

目的了解与弥漫性结缔组织病相关的肺间质病变（CTD—ILD）的发生率、影像学特征及其与病种和临床指标的相关性。方法回顾分析我院几种弥漫性结缔组织病患者（共412例）资料，X线胸片检查或有肺部临床症状患者作胸部高分辨CT（HRCT）进一步检查确诊。了解ILD在不同弥漫性CTD中的发生率，对比X线与HRCT的差异，统计常用CT分类在本组疾病中的分布比例．探讨CT分类与不同病种或临床特征之间的关系。结果在412例弥漫性结缔组织病中，HRCT诊断的ILD共40例，总发生率为9．7％。其中皮肌炎／多肌炎为25％，硬皮病为23．8％，原发性干燥综合征为9．6％，系统性红斑狼疮为6．3％。胸片与CT符合率40％，胸片误诊漏诊率60％。ILDCT分类以两种病变共存最多见，其中毛玻璃样变占40．1％，实变影32．8％，网格影16．4％，蜂窝状影9．8％。呼吸道症状和补体C3，C4水平在毛玻璃样变组及实变影组；C反应蛋白（CRP）水平在蜂窝状影和实变影组较其他对照组明显高，它们之间差异有统计学意义（P〈0．05）。结论HRCT发现CTD—ILD的敏感性及准确性明显优于X线，其中以毛玻璃及实变影最多见；影像学改变与呼吸道症状及炎症指标有一定关系，尽早进行HRCT的检查有助ILD的早期诊断及预后判断。     

结缔组织病肺间质病变的临床、影像和病理诊断

目的了解结缔组织病肺间质病变（connective tissue disease-interstitial lung disease,CTD-ILD）的临床、影像及病理学特征,探讨CTD-ILD影像学与病理类型间的相关性。方法通过高分辨计算机体层成像（high-resulution computed tomography,HRCT）技术了解132例弥漫性结缔组织病合并肺间质病变患者的肺间质病变影像学特点及分型,经皮肺穿刺活检技术进行肺间质病变病理分型。结果与系统性红斑狼疮（systemic lupuserythematosus,SLE）、多发性肌炎（polymyositis,PM）、系统性硬化症（systemic sclerosis,SSc）、类风湿关节炎（rheumatoid arthritis,RA）相比,原发性干燥综合征（primary Sj觟gren＇s syndrome,pSS）、皮肌炎（dermatomysitis,DM）、血管炎肺间质病变的呼吸道症状更明显。从影像学上看CTD-ILD表现多种多样：SLE以磨玻璃影多见,pSS以实变影多见,DM、PM、血管炎以实变影及毛玻璃影多见,SSc和RA以网格影多见。不同疾病肺间质病理类型有显著性差异,SLE、pSS、DM以非特异性肺间质炎（nonspecific interstitial pneumonia,NSIP）为主,SSc以寻常型肺间质炎（usual interstitial pneumonia,UIP）为主。病理类型NISP在影像学上多表现为毛玻璃样实变病变,UIP则更多表现为网格样蜂窝样病变,与其他影像学表现比较有统计学差异（P〈0.05）。用力肺活量和一氧化碳弥散能力下降在病种、影像学及病理改变上均无统计学差异。结论HRCT对诊断CTD-ILD有高敏感性及特异性,影像学改变可帮助临床医生推测其组织病理类型,便于随访和疗效的评价,减少肺活检的危险,是目前诊断CTD-ILD的重要手段。     

北京地区患严重急性呼吸综合征医务人员的胸部高分辨率CT与肺功能随访分析

目的分析医务人员患严重急性呼吸综合征(SAILS)后肺部恢复情况。方法对北京地区2003年在医疗工作岗位上感染SAILS病毒发病的456例医务人员，于2003年12月至2004年2月进行胸部高分辨率CT(HRCT)和肺功能测定。结果在406例进行肺通气功能检测的患者中，31例(7．64％)异常；404例进行肺弥散功能检测，其中165例(40．84％)异常；434例进行胸部HRCT检查，151例(34．79％)异常，表现为磨玻璃密度阴影、网格状阴影和胸膜下弧线影等；395例同时接受肺弥散功能检测和胸部HRCT检查，55例(13．92％)两项均异常，且HRCT显示双肺病变典型者的肺弥散功能较HRCT正常者有显著的下降，两组差异有统计学意义。结论SAILS病毒感染的医务人员在康复出院后尚有部分患者遗留肺功能降低、胸部HRCT异常等问题，提示肺部损伤为慢性改变，应对此类患者继续随访。     

高分辨CT对结缔组织病所致肺间质病变的诊断价值

目的评价胸部高分辨率CT(HRCT)对早期发现结缔组织病所致肺间质病变的诊断价值。方法选择南京红十字医院及安徽医科大学附属医院2004年7月至2005年6月门诊及住院的系统性红斑狼疮患者20例、类风湿关节炎及干燥综合征患者共16例,行X线胸片及常规螺旋CT全胸扫描,运用HRCT对肺部扫描,进行对比分析。结果肺HRCT示21例(58.3%)患肺间质病变(ILD),常规螺旋CT示11例(30.6%)ILD,而X线全胸片仅4例(11.1%)示ILD。HRCT可见如蜂窝状阴影、胸膜下线影、小叶间隔增厚、毛玻璃样变等常规CT及X线胸片下无法观察到的征象。结论HRCT能充分显示肺间质纤维化的各种征象,对结缔组织病引起的肺间质病变的早期诊断有重要意义。     

2型糖尿病患者胸部CT表现及短期随访观察

目的 探讨T2DM患者胸部CT表现及随访肺内病变变化与临床的关系。方法回顾性观察60例T2DM患者胸部CT图像,用CT视觉评分方法定量分析肺内病变,并与CT复查图像进行对照,分析肺内病变变化特征与临床的关系。结果60例T2DM患者中胸部CT表现正常9例（15％）、磨玻璃样影31例（52％）、小叶间隔增厚32例（53％）,多于胸膜下线8例（13％）、支气管血管束增厚3例（5％）、小叶内间质增厚3例（5％）等病变（X2=76．979,P＜0．01）。肺内病变具有周围分布、下肺分布为主的特点。复查后仅14例磨玻璃样病变有不同程度吸收,与磨玻璃影积分值无变化组相比,磨玻璃影积分值变化组病程较短（t=2．485,P=O．023）,HbA1c较低（u=2．831,P=0．015）。结论CT及其随访检查的半定量分析肺内病变,对评估糖尿病肺损害的短期变化及与临床的关系有一定价值。     

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.     

肺真菌病59例病原学和影像学分析

目的：探讨肺真菌病的病原学分布和影像学特征。方法收集肺真菌病59例,均经支气管镜、经皮肺穿刺活检或手术切除送病理学确诊,分析其病原学分布和影像学特征。结果59例病理学确诊肺真菌病患者中,肺曲霉病24例（40．7％）,肺隐球菌病24例（40．7％）,肺毛霉病5例（8．5％）,肺念珠菌病4例（6．8％）,组织胞浆菌病2例（3．4％）,合并放线菌肺炎1例（1．7％）。胸部影像学改变包括肺部肿块23例（39．0％）,渗出性病变23例（39．0％）,结节8例（13．6％）,支气管肿物3例（5．1％）,空洞病变1例（1．7％）,弥漫性病变1例（1．7％）。误诊为肺炎12例（20．3％）,肺结核7例（11．9％）,肺癌4例（6．8％）。结论病理学确诊的肺真菌病以肺曲霉病和肺隐球菌病为多见,影像学表现主要以肺部肿块影和渗出性病变为主。肺真菌病影像学表现多种多样、缺乏特征性,诊断应尽早取得病理学依据。     

类风湿性关节炎累及胸部的CT诊断价值

目的探讨类风湿性关节炎（rheumatoid arthritis,RA）合并肺间质病变的临床特点及CT表现。方法回顾我院2006年10月—2010年12月36例RA并发肺间质病变的临床及CT改变。结果 36例患者胸部X线平片主要表现为：两肺纹理增粗,肺间质及胸膜改变不明显;常规CT扫描表现为：肺间质的改变及胸膜病变;肺高分辨CT〈HRCT〉可见到更丰富的影像学改变,主要有肺磨玻璃样变,小蜂窝样囊腔改变,小叶间隔增厚,网格影及胸膜下微结节和小结节。结论 RA引起的肺部换害,特别是间质性肺病,由于早期症状还明显,在临床工作中易被忽视或延误诊断,因此有必要提高临床医生和患者对RA所致肺损害的认识,提示临床医生对相关因素的RA患者尽早作胸部CT扫描,特别是肺HRCT检查,以早期了解RA浸润肺部的程度及估计预后。     

雷诺现象为首发表现的自身免疫性疾病疾病谱及临床特点

目的观察雷诺现象为首发临床表现的自身免疫性疾病疾病谱,以及其临床受累脏器的特点。方法选择2012年10月至2013年3月北京协和医院风湿免疫科门诊以雷诺现象为首发临床表现的自身免疫性疾病患者。除实验室常规检查外,所有患者均行胸部高分辨CT及超声心动图检查。结果共135例患者纳入研究,男9例,女126例,以系统性红斑狼疮（45．9％）、系统性硬化症（20．7％）及未分化结缔组织病（15．6％）最常见。系统受累以皮肤黏膜（97例,71．9％）、关节（86例,63．7％）及肺部受累（72例,53．3％）多见。其中合并肺间质病变者63例（46．7％）,以系统性红斑狼疮（29例,46．8％）、系统性硬化症（17例,60．O％）及混合性结缔组织病（6例,83．3％）多见;合并肺动脉高压者37例（27．4％）,以系统性红斑狼疮（16例,25．8％）、系统性硬化症（10例,35．7％）、未分化结缔组织病（4例,19．7％）及原发性干燥综合征（2例,28．6％）多见。结论雷诺现象可以是多种自身免疫性疾病的早期表现,尤其是系统性红斑狼疮及系统性硬化症。合并雷诺现象的患者更易出现肺部受累,尤其是肺间质病变及肺动脉高压,应重视在发病初期进行筛查。胸部高分辨cT及超声心动图检查分别是早期筛查肺间质病变及肺动脉高压的简便有效的方法。     

352例结缔组织病并间质性肺疾病临床特点分析

目的本研究通过比较分析结缔组织病并间质性肺疾病（connectivetissuediseaseandinterstitiallungdisease,CTD-ILD）患者的临床特点,旨在提高临床医师对CTD-ILD疾病的认识水平。方法收集并回顾性分析2011年1月1日至2012年12月31日于郑州大学第一附属医院初诊为CTD-ILD的患者的临床特征及影像学特点。结果CTD-ILD患者352例,男女发病比例为1：2．67,平均发病年龄（53．07±13．65）岁。CTD-ILD患者全身症状发生率高于呼吸系统症状。CTD-ILD的高分辨率CT表现多为对称性磨玻璃影、网格状、胸腔积液等改变,主要累及中肺和下肺。结论CTD-ILD多发生于育龄期女性,部分肺部病变首发于CTD症状之前,并可独立进展。高分辨率CT有助于ILD的早期诊断及治疗方案制定。     

Prevalence of interstitial lung involvement in patients with connective tissue diseases assessed with high-resolution computed tomography

OBJECTIVES: To assess the prevalence of interstitial lung disease (ILD) in patients with different forms of connective tissue disease (CTD) using non-invasive procedures including high-resolution computed tomography (HRCT) and to evaluate the relationship between the imaging and functional status of the patients. METHODS: Eighty-one subjects with CTD (47 inpatients and 34 outpatients) were evaluated with pulmonary function tests (PFT) and radiological investigations. The extent and severity of lung disease was quantified with an HRCT scoring system previously used in patients with systemic sclerosis (SSc). Interstitial lung involvement was defined as predominantly fibrotic or inflammatory based on HRCT abnormalities. RESULTS: HRCT abnormalities suggestive of ILD were observed in 69 patients (85.1%), whereas PFT and plain radiograph alterations occurred less frequently (40.7%). The most frequent HRCT abnormalities were septal/subpleural lines and ground-glass appearance whereas lesions consistent with advanced fibrosis were observed in a minority of patients. The HRCT score was higher in patients with abnormal PFT (p<0.001). Thirty-five patients had predominant fibrosis and 34 patients predominantly inflammatory abnormalities. A score of 10 points represented the best compromise between sensitivity and specificity in predicting functional impairment. CONCLUSIONS: A high prevalence of ILD was found based on HRCT abnormalities. However, HRCT scans characterized by minor abnormalities have poor specificity for clinically significant disease and functional findings should also be considered. The large number of patients with predominantly inflammatory HRCT abnormalities suggests that many cases of ILD may be diagnosed in a relatively early stage of the disease.     

65例间质性肺疾病临床特征分析

目的分析总结我院呼吸科间质性肺疾病（ILDs）的临床特点。方法对我院呼吸科间质性肺疾病患者的临床资料进行分析总结。结果 1年间共收治ILDs患者65例,临床表现主要为活动后气短（89.23%）,17例肺功能检查DLco均下降,胸部CT可有不同的表现,磨玻璃影（74.55%）,网格影（38.18%）,蜂窝肺（29.09%）,结节影（14.55%）,实变影（14.55%）,支气管扩张影（27.27%）,多以双下肺和胸膜下为著,血气分析以低氧血症（41.27%）为主,P（A-α）O2均有不同程度的升高,支气管肺泡灌洗液细胞计数以中性粒细胞比例增高为主,肺动脉压力亦有不同程度升高。结论间质性肺疾病的临床表现、实验室检查、影像学与肺功能特征等可做出初步诊断。     

Connective tissue diseases and the liver

Connective tissue diseases such as systemic lupus erythematosus (SLE), rheumatoid arthritis, Sj?gren's syndrome, and scleroderma are systemic disorders that may have an autoimmune basis. The system manifestations vary, and there is frequent overlap among the syndromes. Liver involvement in patients with connective tissue diseases has been well documented but is generally considered rare. Although advanced liver disease with cirrhosis and liver failure is rare in patients with connective tissue diseases, clinical and biochemical evidence of associated liver abnormalities is common. Previous treatment with potentially hepatotoxic drugs or coincident viral hepatitis has usually been implicated as the main causes of liver disease in patients with connective tissue diseases. However, even after careful exclusion of these etiologies, the question remains whether to classify the patient as having a primary liver disease with associated autoimmune, clinical, and laboratory features or as having liver disease as a manifestation of generalized connective tissue disease. The main example of this pathogenetic dilemma is autoimmune hepatitis and SLE-associated hepatitis, which have been regarded as two different entities, although they have features in common of autoimmune syndromes. Several clinical and histopathologic features have been used to discriminate autoimmune hepatitis from SLE, a relevant diagnostic exercise because complications and therapy are quite different. Although hepatic steatosis and abnormal results on biochemical liver function tests are the most common hepatic abnormalities associated with connective tissue diseases, other less frequent abnormalities have been noted, such as nodular regenerative hyperplasia, portal vein obliteration and portal hypertension, features of primary biliary cirrhosis, and rarely portal fibrosis with abnormal lobular architecture. Vascular disorders of the liver also have been described, such as Budd-Chiari syndrome. Histologic assessment may reveal a variety of subclinical liver diseases. The aim of this contribution is to review the current published data regarding liver involvement in connective tissue diseases.     

Computed tomographic imaging in connective tissue diseases

The connective tissue diseases are a group of autoimmune mediated disorders characterized by multiorgan damage. High-resolution computed tomography (HRCT) plays an important role in identifying patients with respiratory involvement. Although there is some overlap, typical HRCT appearances are increasingly recognized for each of the major connective tissue diseases and are described in detail in this review (rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, Sj?gren syndrome, polymyositis/dermatomyositis, mixed connective tissue disease). The clinical utility of HRCT is discussed with respect to the detection of disease, diagnosis, staging of disease severity, prognostic evaluation, and monitoring of disease progression. The difficult problem of establishing the clinical significance of CT abnormalities is also addressed.     

经纤维支气管镜肺活检对弥漫性间质性肺疾病的诊断价值

目的探讨经纤维支气管镜肺活检（TBLB）对弥漫性间质性肺疾病（D ILD）的诊断价值。方法报道168例D ILD患者,其中行TBLB病理检查确诊45例（26.79%）,分析患者性别、病理诊断构成比及误诊情况等。结果 45例患者中,误诊为非特异性肺炎8例,肺结核6例,肺癌5例,肺淋巴瘤4例,肺动脉高压3例,气道中心纤维化2例;女性患者明显多于男性（27 vs 18,P〈0.01）。D ILD患者中特发性间质性肺炎最多（32例）,其次是结缔组织病相关D ILD（8例）、结节病（5例）。结论 TBLB是诊断D ILD的有效手段,对D ILD的鉴别诊断和分类诊断有重要意义。     

Lung fibrosis quantified by HRCT in scleroderma patients with different disease forms and ANA specificities

OBJECTIVE: To define the prevalence of interstitial lung fibrosis in systemic sclerosis (SSc) and its relationship with the different clinical forms of disease and ANA specificities. METHODS: Fifty patients with SSc were submitted to pulmonary high resolution computed tomography (HRCT). Lung abnormalities were evaluated according to Warrick's score that considers both the severity and the extent of fibrotic lesions. RESULTS: Pulmonary HRCT abnormalities were observed in 84% of SSc patients. Ground glass aspects (60%), irregular pleural margins (56%) and septal/subpleural lines (68%) were the most common lesions. The distribution of these abnormalities favoured the posterior basilar segments of both lungs. HRCT findings were significantly more frequent in males and in patients with the cutaneous diffuse form of SSc and with the specific antibody anti-Scl70. CONCLUSIONS: HRCT is a very useful method for the diagnosis of interstitial lung fibrosis in SSc. Warrick's score permits to quantify the HRCT findings and to evaluate their relationship with the disease clinical forms and ANA specificities.