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。     

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。     

结缔组织病相关间质性肺病的诊治策略

结缔组织病(connective tissue disease,CTD)是一组全身性自身免疫性疾病,病变累及多种脏器。由于肺和胸膜均富含胶原、血管等结缔组织,因此 CTD 大多可以损伤肺和胸膜等呼吸系统多个器官,包括：呼吸肌、胸膜、肺血管、气道、肺实质和肺间质,且部分患者呼吸道表现为首发症状。间质性肺病(interstitial lung disease,ILD)在 CTD 中十分常见,发生率在数个 CTD 病种中超过50%。与之相对应,15%~30%初诊为特发型间质性肺炎(idiopathic interstitial pneumonia,IIP)的患者最终被确认符合 CTD-ILD 诊断。ILD 是导致 CTD 患者死亡的重要原因之一。CTD 包括包括系统性红斑狼疮(SLE)、类风湿性关节炎(RA)、原发性干燥综合征(pSS)、多发性肌炎(PM)、皮肌炎(DM)、系统性硬化(SSc)和混合性 CTD (MCTD)等。本文就 CTD-ILD 的诊治策略作一简要综述。     

CXCL4在系统性硬化病患者血清中的表达及临床意义研究

<正>系统性硬化病(SSc)是一种以皮肤及内脏器官纤维化为主要特点的结缔组织病(CTD),发病率2.3~22.8/100万,肺间质病变(ILD)及肺动脉高压(PAH)为其主要的致死因素。SSc发病机制尚不明确,普遍认为血管、免疫系统及纤维化过程之间复杂的相互作用及基因易感性在SSc发病中起重要作用~([1])。CXC趋化因子配体4(CXCL4)主要表达于微血     

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。     

结缔组织病合并肺动脉高压及肺间质病变的肺功能分析

目的 比较和分析不同结缔组织病(CTD)相关肺部病变患者组间各项肺功能指标的差异.探讨肺功能检查对于评价CTD肺部病变性质和程度的临床意义.方法 前瞻性纳入CTD肺脏受累患者,根据肺部病变性质分为3组:CTD合并肺动脉高压组(CTD-PAH)29例、合并肺间质病变组(CTD-ILD))35例、合并PAH及ILD组(CTD-PAH+ILD)16例;另纳入无肺部病变的CTD患者34例作为对照组.比较各组间肺总量占预计值百分比(TLC%)、用力肺活量占预计值百分比(FVC%)、第1秒用力呼气量占预计值百分比(FEV_(1.0)%)、FEV_(1.0)/FVC、一氧化碳弥散量占预计值百分比(DLco%)、弥散率(DLco/VA)等主要肺功能指标的差异.结果 共114例患者纳入本研究,以女性多见,平均年龄35～39岁,易合并肺部病变的CTD依次为:混合性结缔组织病(MCTD)、系统性硬化症(SSc)、系统性红斑狼疮(SLE)和原发性干燥综合征(pSS).CTD-PAH组、CTD-ILD组和CTD-PAH+ILD组分别有10%、29%和46%的患者出现TLC下降;分别有50%、36%和71%的患者出现FVC%下降;分别有54%、47%和71%的患者出现FEV_(1.0)%下降;分别有100%、82%和100%的患者出现DLco%下降.多组间样本比较分析发现TLC%、FVC%、FEV_(1.0)%、DLco%在CTD对照组与CTD合并各肺部病变组之间的差异均有统计学意义(P＜0.05),而在CTD-ILD组与CTD-PAH组间差异均无统计学意义.TLC%在CTD-PAH组大于CTD-PAH+ILD组[(89±15)%与(79±12)%,P＜0.05)];FVC%在CTD-PAH组或CTD-ILD组均大于CTD-PAH+ILD组[(81±13)%、(80±16)%与(65±22)%,P＜0.05)].结论 肺功能检查对于筛查CTD合并的各种肺部病变具有临床应用价值,常表现为限制性通气功能障碍和弥散功能障碍,但从单次数值上无法鉴别CTD肺部病变种类(PAH与ILD).     

结缔组织病相关间质性肺病与自身抗体的相关性研究进展

间质性肺疾病(ILD)是结缔组织病(CTD)的重要并发症之一,结缔组织病相关间质性肺病(CTD-ILD)的发病率和病死率较高,并且早期确诊有一定困难,CTD-ILD与自身抗体相关性的研究对其预防、早期确诊、指导治疗及预后极为重要.为了便于临床能正确诊断和及时治疗CTD-ILD,现将常见于CTD的自身抗体及CTD患者发生ILD的临床特点进行综述.     

肺超声在结缔组织病相关间质性肺疾病诊断和治疗监测中的价值

肺是CTD的常见受累脏器, 间质性肺疾病(ILD)是重要的病变类型, 影响患者的预后和生存。高分辨CT(HRCT)是目前诊断和评价ILD严重程度的首选影像学技术, 但在某些特殊人群和临床情况的应用中受到一定的限制。肺超声技术无创、便捷, 因此本文就肺超声在CTD-ILD的表现和价值进行综述。     

结缔组织病相关性间质性肺疾病的诊治进展

结缔组织病（CTD）是一组累及全身多系统的自身免疫病,合并间质性肺疾病（ILD）较为常见,导致肺功能障碍、低氧血症,严重影响患者的生活质量和预后。不同CTD显示出异质性的ILD类型。CTD-ILD常见的影像学和组织病理学类型是非特异性间质性肺炎、机化性肺炎和普通型间质性肺炎。大部分CTD-ILD表现为缓慢进展的肺损伤,少数患者短期内急剧进展且预后不良。CTD-ILD目前主要应用糖皮质激素和／或免疫抑制剂治疗,生物制剂显示出临床疗效,尚需随机临床试验证实。部分ILD患者具有自身免疫病的特征,不足以诊断为某种特定的CTD,应予以密切随访。     

特异性抗膜突蛋白抗体在结缔组织病肺部受累早期诊断中的意义

目的 探讨特异性的自身抗体对结缔组织病(CTD)相关肺部损伤的临床意义.方法 以重组膜突蛋白作为抗原,应用酶联免疫吸附试验(ELISA)和免疫印迹法检测40例系统性硬化症(SSc)患者和38例混合性结缔组织病(MCTD)患者外周血抗膜突蛋白抗体.比较不同肺脏受累的CTD患者之间抗膜突蛋白抗体的差异.结果 ELISA和免疫印迹法检测发现21例(52%)SSc患者和15例(39%)MCTD患者抗膜突蛋白抗体阳性.CTD合并肺脏受累组抗膜突蛋白抗体的滴度均显著高于无肺脏受累组(0.23±0.14与0.12±0.35,P=0.001).在肺功能检查方面,抗膜突蛋白抗体阳性患者组与阴性患者组比较,肺总量[(82±10)%与(90±14)%,P=0.027],用力肺活量[(76±13)%与(85±17)%,P=0.040],一氧化碳弥散量[(58±16)%与(72±23)%,P=0.014]均显现显著降低.结论 抗膜突蛋白抗体在SSc和MCTD患者中具有较高的阳性率,并与CTD合并肺脏受累显著相关,可能早期提示结缔组织病肺脏损害.     

Comparison of disease progression and mortality of connective tissue disease-related interstitial lung disease and idiopathic interstitial pneumonia

OBJECTIVE: To compare disease progression and mortality between idiopathic interstitial pneumonia (IIP) and interstitial lung disease (ILD) due to connective tissue diseases (CTD) including scleroderma, rheumatoid arthritis, systemic lupus, polymyositis, dermatomyositis, Sj?gren's syndrome, and mixed CTD. METHODS: A case-control study of patients with CTD-ILD (n = 46) and IIP controls (n = 51), seen at the University of Michigan between July 1,1998 and June 30,1999 and followed until March 30, 2002, was conducted. Survival analysis and Cox regression were performed to estimate survival, accounting for demographic and clinical parameters, including pulmonary function tests and high resolution computed tomography (HRCT) diagnosis and scoring. RESULTS: Median followup time was 4.4 person-years. Five-year survival in the IIP group was 51.9% (95% confidence interval [95% CI] 30.8-69.4) versus 43.4% (95% CI 21.1-63.9) in the CTD-ILD group. There were no significant differences among HRCT diagnostic categories between IIP and CTD-ILD. A fibrotic score > or = 2 was associated with decreased survival among the entire group. Age at diagnosis and most recent forced vital capacity were significant predictors of mortality when adjusted for IIP versus CTD-ILD diagnosis, sex, and interstitial score. CONCLUSION: Contrary to expectation, CTD-ILD compared with IIP appears to be associated with a worse prognosis when adjusted for age. A higher fibrotic score is suggestive of decreased survival.     

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.     

Radiographic fibrosis score predicts survival in systemic sclerosis‐associated interstitial lung disease

Background and objective

Interstitial lung disease (ILD ) is a common pulmonary manifestation of systemic sclerosis (SSc ). It is unknown whether radiographic fibrosis score predicts mortality in SSc ‐associated ILD (SSc‐ILD ). We retrospectively analysed patients with SSc‐ILD to evaluate whether radiographic fibrosis score was a useful predictor of mortality.

Methods

We identified SSc‐ILD patients evaluated at Kurashiki Central Hospital (Japan) from 2006 to 2016, and radiographic fibrosis scores based on the extent of reticulation and honeycombing on high‐resolution computed tomography (HRCT ) scanning were calculated by manually tracing around each fibrotic area. Independent predictors of overall survival were determined using the Cox proportional hazards model.

Results

The study included 48 patients, of whom 19 had usual interstitial pneumonia on HRCT . The median follow‐up period was 56.6 months, and over the follow‐up period 15 patients died. The 5‐year survival was 72.4%. In the multivariate analysis, radiographic fibrosis score, age, being male and forced vital capacity were independently associated with an increased risk of death, while HRCT pattern was not.

Conclusion

A high radiographic fibrosis score was a poor prognostic factor in SSc‐ILD . More widespread fibrosis was associated with an increased risk of death, independent of HRCT pattern.

     

Bronchoalveolar lavage fluid and progression of scleroderma interstitial lung disease

Introduction: So far no clinical or experimental evidences clearly explain how and which systemic sclerosis (SSc) patients will experience a functional and radiological progression of interstitial lung disease (ILD). Objectives: The aim of the study was to investigate whether any bronchoalveolar lavage fluid (BALF) characteristic, compared with clinical, functional and radiological parameters, is associated with the risk of progression of ILD and worse survival in SSc patients. Methods: Lung involvement was evaluated in 110 consecutively examined SSc patients with pulmonary function tests (PFTs) and high‐resolution computed tomography (HRCT); 73 patients with evidence of ILD on HRCT underwent BAL. The progression of ILD was evaluated with PFTs and HRCT after 1‐year follow‐up. A 36‐month survival analysis was assessed. Results: ILD patients with alveolitis had a higher risk to have restrictive lung disease and honeycombing, to experience a worsening in honeycombing score or to develop honeycombing. ILD progression was associated with the evidence of honeycombing on HRCT, with the presence of eosinophils, with an inverted CD4/CD8 ratio and with a higher CD19 percentage count in the BALF or with a positive BALF microbiological culture. The patients with ILD had a worse overall survival. The diffuse disease was the only independent risk factor of overall mortality, and the extent of honeycombing on HRCT was the only independent risk factor of lung disease‐related mortality. Conclusion: Our study suggests the importance of evaluating ILD with HRCT and BAL in order to characterize the risk factors of SSc lung involvement progression. Please cite this paper as: De Santis M, Bosello SL, Peluso G, Pinnelli M, Alivernini S, Zizzo G, Bocci M, Capacci A, La Torre G, Mannocci A, Pagliari G, Varone F, Pistelli R, Danza FM and Ferraccioli G. Bronchoalveolar lavage fluid and progression of scleroderma interstitial lung disease. Clin Respir J 2012; 6; 9–17.     

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。     

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。     

不容忽视的结缔组织病并发肺间质病变

肺间质病变（ILD）是与多种结缔组织病（CTD）相关的一类疾病。CTD-ILD病理类型复杂,影像学表现各异,临床治疗困难,是引起CTD患者死亡的重要原因。本文综述了常见结缔组织病并发间质性肺病的组织病理、影像学、临床特点以及相关的治疗进展。     

Experience of Mycophenolate Mofetil in 10 Patients With Autoimmune-Related Interstitial Lung Disease Demonstrates Promising Effects

BackgroundInterstitial lung disease (ILD) is a frequent manifestation of connective tissue disease (CTD), especially systemic sclerosis (SSc), polymyositis-dermatomyositis, and rheumatoid arthritis. ILD related to CTDs heralds a poor prognosis and is associated with high mortality and 60% of patients have evidence of ILD. Cyclophosphamide (CYC) is extensively used in SSc ILD with moderate initial response but a poor long-term outcome, and is associated with significant toxicity.ResultsMycophenolate mofetil (MMF) was administered to 10 patients with autoimmune-related ILD: 4 with SSc, 3 with rheumatoid arthritis, 2 with polymyositis, and 1 with systemic lupus erythematosus and Sjögren syndrome. Five patients received remote CYC infusion. Ten patients had improvement in alveolitis, symptoms (cough, dyspnea, and chest discomfort), perceived quality of life and activity levels. Four of 5 patients discontinued oxygen. Two of 8 repeat high-resolution computed tomography improved, 6 stabilized, none worsened. Pulmonary function testing in 1 of 9 patients showed worsening, 3 with improvement and 5 stabilized. Serial echocardiograms revealed no new pulmonary arterial hypertension and no worsening of preexisting pulmonary arterial hypertension. Very importantly, averaged prednisone dose decreased from 58 to 1.4 mg without worsening.ConclusionMMF is safe, well tolerated, and allows reduction or discontinuation of prednisone without worsening of symptoms or objective progression of disease. MMF is less toxic and its targeted antifibrotic properties make it a potentially more effective agent than CYC that may supplant it as a first-line agent or provide sensible post-CYC maintenance or synergistic strategy in the treatment of CTD-ILD.