Lung disease, interstitial

肺部疾病,间质
  • 文章类型: Journal Article
    认知和身体限制在慢性肺病患者中很常见,但是它们与身体功能和日常生活活动的相互作用并没有得到很好的表征。了解这些相互作用和潜在的贡献者可以提供有关残疾的见解,并实现更量身定制的康复策略。
    结果:本综述总结了为期2天的患者伴侣会议,临床医生,研究人员,和肺部关联,讨论慢性肺部疾病患者的认知和身体功能之间的相互作用。本报告涵盖四个方面:1)慢性肺病患者的认知-身体限制,2)认知评估,3)优化认知和运动控制的策略,4)未来的研究方向。认知和身体损伤对生活质量和日常功能有多重影响。与会者承认有必要进行标准化的认知评估,以补充慢性肺病患者的身体评估。呼吸困难,疲劳,和年龄被认为是认知的重要因素,可以影响运动控制和日常身体功能。强调肺康复是一种多学科策略,可以通过神经可塑性改善呼吸和肢体运动控制。并有可能改善身体机能和生活质量。
    结论:人们一致认为,慢性肺病患者的认知功能和呼吸困难的认知干扰会导致运动控制障碍,从而对日常功能产生负面影响。可以通过肺康复来改善。会议提出了与慢性肺病患者的认知-身体相互作用有关的几个关键研究问题。
    UNASSIGNED: Cognitive and physical limitations are common in individuals with chronic lung diseases, but their interactions with physical function and activities of daily living are not well characterized. Understanding these interactions and potential contributors may provide insights on disability and enable more tailored rehabilitation strategies.
    RESULTS: This review summarizes a 2-day meeting of patient partners, clinicians, researchers, and lung associations to discuss the interplay between cognitive and physical function in people with chronic lung diseases. This report covers four areas: (1) cognitive-physical limitations in patients with chronic lung diseases; (2) cognitive assessments; (3) strategies to optimize cognition and motor control; and (4) future research directions. Cognitive and physical impairments have multiple effects on quality of life and daily function. Meeting participants acknowledged the need for a standardized cognitive assessment to complement physical assessments in patients with chronic lung diseases. Dyspnea, fatigue, and age were recognized as important contributors to cognition that can affect motor control and daily physical function. Pulmonary rehabilitation was highlighted as a multidisciplinary strategy that may improve respiratory and limb motor control through neuroplasticity and has the potential to improve physical function and quality of life.
    CONCLUSIONS: There was consensus that cognitive function and the cognitive interference of dyspnea in people with chronic lung diseases contribute to motor control impairments that can negatively affect daily function, which may be improved with pulmonary rehabilitation. The meeting generated several key research questions related to cognitive-physical interactions in individuals with chronic lung diseases.
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  • 文章类型: Journal Article
    目的:本研究旨在探讨肺癌合并间质性肺疾病(ILD)患者行常规分割胸部放疗后发生严重放射性肺损伤(RILI)的预测因素。
    方法:该研究包括2010年至2021年在两个三级中心连续接受胸部放疗治疗的肺癌患者。使用美国国家癌症研究所通用术语标准5.0版对RILI严重程度进行分级,重度RILI定义为毒性等级≥4级,症状性RILI定义为≥2级。绝对中性粒细胞计数(ANC),绝对淋巴细胞计数(ALC),放疗开始前4周内收集C反应蛋白。中性粒细胞-淋巴细胞比率(NLR)计算为ANC/ALC。中位随访时间为9(范围,6-114)个月。
    结果:在54例患者中,22人(40.7%)患有严重的RILI。在多元逻辑回归分析中,高预处理ANC(p=0.030,OR=4.313),预处理NLR(p=0.007,OR=5.784),和ILD严重程度(p=0.027,OR=2.416)是严重RILI的显著预测因子。剂量学因素与严重RP无关。患有严重RILI的患者的总体生存率明显低于没有RILI的患者,1年累计总生存率为7.4%和62.8%,分别。
    结论:预处理血液NLR,ANC,ILD严重程度与严重RILI相关。严重RILI患者的总体生存率很低。
    This study aimed to investigate the predictive factors of severe radiation-induced lung injury (RILI) in patients with lung cancer and coexisting interstitial lung disease (ILD) undergoing conventionally fractionated thoracic radiotherapy.
    The study includes consecutive patients treated with thoracic radiotherapy for lung cancer at two tertiary centers between 2010 and 2021. RILI severity was graded using the National Cancer Institute Common Terminology Criteria version 5.0, with severe RILI defined as toxicity grade ≥4, and symptomatic RILI as grade ≥2. The absolute neutrophil count (ANC), absolute lymphocyte count (ALC), and C-reactive protein were collected within 4 weeks before starting radiotherapy. Neutrophil-lymphocyte ratios (NLR) were calculated as ANC/ALC. The median follow-up was 9 (range, 6-114) months.
    Among 54 patients, 22 (40.7 %) had severe RILI. On multivariate logistic regression analysis, high pretreatment ANC (p = 0.030, OR = 4.313), pretreatment NLR (p = 0.007, OR = 5.784), and ILD severity (p = 0.027, OR = 2.416) were significant predictors of severe RILI. Dosimetric factors were not associated with severe RP. Overall survival was significantly worse for patients with severe RILI than those without, with 1-year cumulative overall survival rates of 7.4 % and 62.8 %, respectively.
    Pretreatment blood NLR, ANC, and ILD severity were associated with severe RILI. Overall survival was dismal for patients with severe RILI.
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  • 文章类型: Journal Article
    目的:根据FleischnerSociety的定义,评估自动量化间质性肺异常(ILA)在可切除的非小细胞肺癌(NSCLC)患者中的预后意义。
    方法:回顾性纳入2015年1月至2019年12月接受肺叶切除术或肺切除术治疗NSCLC的患者。使用市售的基于深度学习的ILA自动定量软件分析术前CT扫描。根据弗莱施纳学会的量化结果和定义以及多学科的讨论,患者分为正常,ILA,和间质性肺病(ILD)组。
    结果:在1524名患者中,87(5.7%)和20(1.3%)患者患有ILA和ILD,分别。两个ILA(HR,1.81;95%CI:1.25-2.61;p=.002)和ILD(HR,5.26;95%CI:2.99-9.24;p<.001)组无复发生存率(RFS)较差。随着疾病严重程度的增加,总生存期(OS)降低(ILA组的HR2.13[95%CI:1.27-3.58;p=.004]和ILD组的7.20[95%CI:3.80-13.62,p<.001])。定量的纤维化和非纤维化ILA成分均与不良RFS(HR,1.57;95%CI:1.12-2.21;p=0.009;和HR,1.11;95%CI:1.01-1.23;p=.03)和OS(HR,1.59;95%CI:1.06-2.37;p=.02;和HR,1.17;95%CI:1.03-1.33;p=0.01)在正常组和ILA组中。
    结论:基于FleischnerSociety定义的ILA自动CT定量可根据疾病类别和定量的纤维化和非纤维化ILA成分预测可切除肺癌患者的预后。
    结论:ILA的定量CT评估为肺癌术后患者提供了预后信息,这有助于考虑积极监测复发,特别是在那些具有较大程度的量化ILA。
    结论:•在1524例可切除的肺癌患者中,1417例(93.0%)患者被归类为正常,87(5.7%)为间质性肺异常(ILA),20例(1.3%)为间质性肺病(ILD)。•ILA和ILD组均与无复发生存率不良相关(风险比[HR],1.81,p=0.002;HR,分别为5.26,p<.001)和总生存率(HR,2.13;p=.004;HR,7.20;p<.001)。•定量的纤维化和非纤维化ILA组分均与正常和ILA组中的无复发存活和总存活相关。
    OBJECTIVE: To assess the prognostic significance of automatically quantified interstitial lung abnormality (ILA) according to the definition by the Fleischner Society in patients with resectable non-small-cell lung cancer (NSCLC).
    METHODS: Patients who underwent lobectomy or pneumonectomy for NSCLC between January 2015 and December 2019 were retrospectively included. Preoperative CT scans were analyzed using the commercially available deep-learning-based automated quantification software for ILA. According to quantified results and the definition by the Fleischner Society and multidisciplinary discussion, patients were divided into normal, ILA, and interstitial lung disease (ILD) groups.
    RESULTS: Of the 1524 patients, 87 (5.7%) and 20 (1.3%) patients had ILA and ILD, respectively. Both ILA (HR, 1.81; 95% CI: 1.25-2.61; p = .002) and ILD (HR, 5.26; 95% CI: 2.99-9.24; p < .001) groups had poor recurrence-free survival (RFS). Overall survival (OS) decreased (HR 2.13 [95% CI: 1.27-3.58; p = .004] for the ILA group and 7.20 [95% CI: 3.80-13.62, p < .001] for the ILD group) as the disease severity increased. Both quantified fibrotic and non-fibrotic ILA components were associated with poor RFS (HR, 1.57; 95% CI: 1.12-2.21; p = .009; and HR, 1.11; 95% CI: 1.01-1.23; p = .03) and OS (HR, 1.59; 95% CI: 1.06-2.37; p = .02; and HR, 1.17; 95% CI: 1.03-1.33; and p = .01) in normal and ILA groups.
    CONCLUSIONS: The automated CT quantification of ILA based on the definition by the Fleischner Society predicts outcomes of patients with resectable lung cancer based on the disease category and quantified fibrotic and non-fibrotic ILA components.
    CONCLUSIONS: Quantitative CT assessment of ILA provides prognostic information for lung cancer patients after surgery, which can help in considering active surveillance for recurrence, especially in those with a larger extent of quantified ILA.
    CONCLUSIONS: • Of the 1524 patients with resectable lung cancer, 1417 (93.0%) patients were categorized as normal, 87 (5.7%) as interstitial lung abnormality (ILA), and 20 (1.3%) as interstitial lung disease (ILD). • Both ILA and ILD groups were associated with poor recurrence-free survival (hazard ratio [HR], 1.81, p = .002; HR, 5.26, p < .001, respectively) and overall survival (HR, 2.13; p = .004; HR, 7.20; p < .001). • Both quantified fibrotic and non-fibrotic ILA components were associated with recurrence-free survival and overall survival in normal and ILA groups.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    目的:比较间质性肺病(ILD)人群的肺CT容积(CTvol)和肺功能检查。然后评估特发性肺纤维化(IPF)与非IPF之间的CTvol损失,并探讨IPF中每年CTvol损失的预后价值。
    方法:我们在一个专家中心进行了一项2005年至2018年连续ILD患者的回顾性研究。使用基于深度学习算法的商业软件自动测量CTvol。在第一组中,强迫肺活量(FVC)之间的斯皮尔曼相关系数(r),总肺活量(TLC),计算CTvol。在第二组中,使用线性回归分析计算每年CTvol损失,并与Mann-Whitney检验进行比较.在最后一组IPF患者中,计算基线和1年之间的CTvol年度减少量,用Youden指数研究3年时主要不良事件的预后价值.计算单变量和对数秩检验。
    结果:总计,对560例患者(4610个CT)进行分析。对于1171个CT,CTvol与FVC(r:0.86)和TLC(r:0.84)相关(p<0.0001)。在408例患者(3332CT)中,在5.03年期间,IPF患者的年CTvol减少中位数为155.7mL,非IPF患者为50.7mL(p<0.0001).在73名IPF患者中,相对每年7.9%的CTvol损失与主要不良事件相关(log-rank,在单变量分析中p<0.0001)(p<0.001)。
    结论:自动肺CT容积可能是评估ILD肺容积损失的肺功能检查的替代或补充生物标志物。
    结论:•肺部CT容积与用力肺活量有很好的相关性,以及总肺活量测量(r分别为0.86和0.84,p<0.0001)。•特发性肺纤维化患者的年CT体积损失中位数明显高于其他纤维化间质性肺病患者(155.7对50.7mL,p<0.0001)。•在特发性肺纤维化中,相对年CT体积损失高于9.4%与2.0年和2.8年的平均生存时间显著减少相关(log-rank,p<0.0001)。
    OBJECTIVE: To compare the lung CT volume (CTvol) and pulmonary function tests in an interstitial lung disease (ILD) population. Then to evaluate the CTvol loss between idiopathic pulmonary fibrosis (IPF) and non-IPF and explore a prognostic value of annual CTvol loss in IPF.
    METHODS: We conducted in an expert center a retrospective study between 2005 and 2018 on consecutive patients with ILD. CTvol was measured automatically using commercial software based on a deep learning algorithm. In the first group, Spearman correlation coefficients (r) between forced vital capacity (FVC), total lung capacity (TLC), and CTvol were calculated. In a second group, annual CTvol loss was calculated using linear regression analysis and compared with the Mann-Whitney test. In a last group of IPF patients, annual CTvol loss was calculated between baseline and 1-year CTs for investigating with the Youden index a prognostic value of major adverse event at 3 years. Univariate and log-rank tests were calculated.
    RESULTS: In total, 560 patients (4610 CTs) were analyzed. For 1171 CTs, CTvol was correlated with FVC (r: 0.86) and TLC (r: 0.84) (p < 0.0001). In 408 patients (3332 CT), median annual CTvol loss was 155.7 mL in IPF versus 50.7 mL in non-IPF (p < 0.0001) over 5.03 years. In 73 IPF patients, a relative annual CTvol loss of 7.9% was associated with major adverse events (log-rank, p < 0.0001) in univariate analysis (p < 0.001).
    CONCLUSIONS: Automated lung CT volume may be an alternative or a complementary biomarker to pulmonary function tests for the assessment of lung volume loss in ILD.
    CONCLUSIONS: • There is a good correlation between lung CT volume and forced vital capacity, as well as for with total lung capacity measurements (r of 0.86 and 0.84 respectively, p < 0.0001). • Median annual CT volume loss is significantly higher in patients with idiopathic pulmonary fibrosis than in patients with other fibrotic interstitial lung diseases (155.7 versus 50.7 mL, p < 0.0001). • In idiopathic pulmonary fibrosis, a relative annual CT volume loss higher than 9.4% is associated with a significantly reduced mean survival time at 2.0 years versus 2.8 years (log-rank, p < 0.0001).
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  • 文章类型: Journal Article
    Objective: To provide reference for clinicians in diagnosis and treatment of antisynthetase syndrome with interstitial pulmonary disease (ASS-ILD) by analyzing the clinical features, imaging features and pulmonary function changes of ASS-ILD patients. Methods: A total of 92 patients with ASS-ILD diagnosed in the Respiratory Center of China-Japan Friendship Hospital from January 2015 to May 2018 were included, clinical manifestations, high-resolution computed tomography (HRCT), pulmonary function test, treatment and outcome were retrospectively analyzed. Results: The average age of the 92 patients was (58.6±12.2) years with a ratio of male to female 1∶1.79. The main types of anti-synthetase antibody were anti-Jo-1 antibody (37 cases, 40.2%) and anti EJ antibody (26 cases, 28.3%). The most common symptoms of ASS-ILD were cough (79 cases, 85.9%), shortness of breath (60 cases, 65.2%), expectoration (54 cases, 58.7%), fever (34 cases, 36.9%), and common signs were craftsman\'s hand (30 cases, 32.6%) and joint pain (23 cases, 25.0%). The most common imaging findings in HRCT were ground-glass opacities(68 cases, 73.9%), reticulations (45 cases, 48.9%), tractive bronchiectasis (40 cases, 43.5%) and consolidation (39 cases, 42.4%). The most common types of ILD were non-specific interstitial pneumonia (NSIP) (63 cases, 68.5%), followed by NSIP-organic pneumonia (OP) (12 cases, 13.0%). The main type of lung function impairment was mild restrictive ventilation dysfunction. There were no significant differences in clinical features, imaging findings and pulmonary function changes in different subtypes of ASS-ILD patients (all P>0.05). Corticosteroids was used in 78 (84.8%) of ASS-ILD patients, and 34 cases (37.0%) were given cyclophosphamide. A total of 91 patients (98.9%) were discharged with improvement and 1 anti-EJ positive patient died. Conclusions: ASS-ILD occurs frequently in middle-aged and old women. Respiratory symptoms, craftsman\'s hands and arthritis are the most common clinical manifestations. The most common imaging types of ILD are NSIP and NSIP-OP. Corticosteroids is commonly used for treatment.
    目的: 总结抗合成酶综合征(ASS)合并间质性肺病(ILD)患者的临床特征。 方法: 回顾性分析2015年1月至2018年5月中日友好医院呼吸中心诊断为ASS-ILD患者92例,对其初诊时的临床表现、高分辨率CT(HRCT)影像学特点、肺功能指标以及治疗与转归进行总结分析。 结果: ASS-ILD患者年龄(58.6±12.2)岁(范围:17~83岁),男女比为1∶1.79,抗合成酶抗体分型以抗Jo-1抗体阳性(37例,40.2%)和抗EJ抗体阳性(26例,28.3%)为主。ASS-ILD最常见的症状是咳嗽(79例,85.9%)、气短(60例,65.2%)、咳痰(54例,58.7%)、发热(34例,36.9%),体征常见技工手(30例,32.6%)及关节疼痛(23例,25.0%)。CT影像学上以磨玻璃影(68例,73.9%)、网格影(45例,48.9%)、牵拉性支气管扩张(40例,43.5%)以及实变(39例,42.4%)常见,ILD类型以非特异性间质性肺炎(NSIP)(63例,68.5%)最多见,其次是NSIP-机化性肺炎(OP)(12例,13.0%)。肺功能改变主要为轻度限制性通气功能障碍。不同抗合成酶抗体亚型的ASS-ILD患者之间临床、影像学及肺功能比较差异均无统计学意义(P值均>0.05)。78例(84.8%)患者应用激素治疗,34例(37.0%)使用环磷酰胺。91例(98.9%)患者好转出院,1例抗EJ阳性患者因呼吸衰竭死亡。 结论: ASS-ILD以中老年女性多发,临床表现以呼吸道症状、技工手、关节炎常见,影像学最常见的类型为NSIP和NSIP-OP,临床治疗常应用激素。.
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  • 文章类型: Journal Article
    Objective: To study the characteristics and clinical significance of pulmonary function test and kerbs von den lungen 6 (KL-6) in anti-synthetase syndrome related interstitial lung disease (ASSD-ILD) and idiopathic pulmonary fibrosis (IPF). Methods: The clinical data of 43 patients with ASSD-ILD (ASSD-ILD group) from May 2015 to May 2017 were collected retrospectively, including 12 males and 31 females, and 34 patients with IPF (IPF group) treated in the First Affiliated Hospital of Zhengzhou University during the same period, including 28 males and 6 females, were also included. The basic information, and the value of pulmonary function test [pulmonary function parameters included the forced vital capacity expressed as percent predicted (FVC%pred), the forced expiratory volume in 1 second expressed as percent predicted (FEV(1)%pred), the ratio of FVC to FEV(1) (FVC/FEV(1)), the peak expiratory flow expressed as percent predicted (PEF%pred), the forced expiratory flow at 25%, 50%, 75% of FVC as percent predicted (FEF(25)%pred, FEF(50)%pred, and FEF(75)%pred), the maximum mid-expiratory flow as percent predicted (MMEF% pred), and the diffusing capacity for carbon monoxide as percent predicted (DLCO% pred)], and serum KL-6 level in ASSD-ILD and IPF were compared. Results: The FEV(1)%pred, FEF(50)%pred, FEF(75)%pred, and MMEF%pred values in ASSD-ILD group were significantly lower than those in IPF group (all P<0.05), while the FVC% pred, FVC/FEV(1), PEF% pred, FEF(25)%pred, and DLCO% pred values in ASSD-ILD group had no significant difference compared with IPF group (all P>0.05). There was no significant difference in serum KL-6 level between ASSD-ILD group and IPF group [(1 169±911) vs (1 210±908) U/ml, t=0.62, P=0.463]. Follow-up analysis showed that the serum KL-6 level of ASSD-ILD patients who died within two years was significantly higher than that of survivors [(2 060±1 168) vs (1 042±858) U/ml, t=2.93, P=0.041]. The serum KL-6 level of patients who died within two years of IPF patients was also significantly higher than that of patients who survived [(1 767±865) vs (1 089±894) U/ml, t=2.53, P=0.026]. The serum KL-6 level in ASSD-ILD group was negatively correlated with FVC%pred (r=-0.43, P=0.004), FEV(1)%pred (r=-0.39, P=0.010) and DLCO% pred (r=-0.41, P=0.006). There was no correlation between serum KL-6 level and pulmonary function test indexes in IPF group (all P>0.05). Conclusions: There is difference in pulmonary function test between ASSD-ILD patients and IPF patients. High serum KL-6 level will be predictive of poor prognosis.
    目的: 探讨肺功能和涎液化糖链抗原-6(KL-6)在抗合成酶综合征合并间质性肺疾病(ASSD-ILD)与特发性肺纤维化(IPF)中的特点及临床意义。 方法: 回顾性收集2015年5月至2017年5月郑州大学第一附属医院诊治的43例ASSD-ILD患者(其中男12例,女31例)(ASSD-ILD组)及34例IPF患者(其中男28例,女6例)(IPF组)的临床资料,比较两组患者基本信息、肺功能结果[包括用力肺活量占预计值的百分比(FVC%预计值)、第1秒用力呼气容积占预计值的百分比(FEV(1)%预计值)、FEV(1)与FVC的比值(FEV(1)/FVC)、呼气峰值流量占预计值的百分比(PEF%预计值)、用力呼出25%、50%、75%肺活量时的瞬间呼气流量占预计值的百分比(FEF(25)%预计值、FEF(50)%预计值、FEF(75)%预计值)、最大呼气中期流量占预计值的百分比(MMEF%预计值)、肺弥散量占预计值的百分比(DLCO%预计值)]及KL-6水平,分析ASSD-ILD与IPF组中肺功能和KL-6的特点及临床意义。 结果: ASSD-ILD组FEV(1)%预计值、FEF(50)%预计值、FEF(75)%预计值、MMEF%预计值均显著低于IPF组(均P<0.05),而ASSD-ILD组FVC%预计值、FEV(1)/FVC、PEF%预计值、FEF(25)%预计值及DLCO%预计值与IPF组相比差异无统计学意义(均P>0.05)。ASSD-ILD组血清KL-6水平与IPF组相比差异无统计学意义[(1 169±911)比(1 210±908)U/ml,t=0.62,P=0.463]。随访分析表明,ASSD-ILD两年内死亡患者血清KL-6水平显著高于存活患者[(2 060±1 168)比(1 042±858)U/ml,t=2.93,P=0.041];IPF两年内死亡患者血清KL-6水平也显著高于存活患者[(1 767±865)比(1 089±894)U/ml,t=2.53,P=0.026]。ASSD-ILD组血清KL-6水平与FVC%预计值(r=-0.43,P=0.004)、FEV(1)%预计值(r=-0.39,P=0.010)及DLCO%预计值(r=-0.41,P=0.006)均呈负相关;IPF组血清KL-6水平与肺功能各项指标均无相关性(均P>0.05)。 结论: ASSD-ILD患者与IPF患者之间肺功能存在差异,高水平血清KL-6提示患者预后较差。.
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  • 文章类型: Journal Article
    Objective: To evaluate the diagnostic value of the serum Krebs von den Lungen-6 (KL-6) for the interstitial lung disease associated with connective tissue diseases (CTD-ILD). Methods: 84 patients with CTD-ILD (CTD-ILD group) and 91 patients with connective tissue disease (CTD group) who visited the department of rheumatology and immunology of People\'s Hospital of Xinjiang Uygur Autonomous Region between January, 2016 and December, 2017 were included. Serum KL-6 levels were measured by commercially available enzyme linked immunosorbent assay (ELISA) kits. Results: The significantly higher levels of KL-6 were determined in the CTD-ILD group than CTD group [1 239 (577, 2 094) vs 152 (89, 280) U/ml] (P<0.001). The optimal cutoff value of serum KL-6 for diagnosis of CTD-ILD was 402 U/ml, and the sensitivity and specificity were 82.1% and 86.8%, respectively. Area Under the Curve (AUC) was 0.905. Logistic regression analysis revealed that elevated KL-6 and decreased Carbon monoxide diffusion capacity were independently correlated with the occurrence of CTD-ILD, the decreased of DLcoSB% (OR=0.928, 95%CI: 0.891-0.968) and increase of KL-6 level (OR=1.005, 95%CI: 1.003-1.007) was the independent risk factor for the occurrence of ILD. Conclusion: The serum KL-6 is an important biomarker for the diagnosis of CTD-ILD and when the level of KL-6 is increased, the ILD should be alert.
    目的: 探讨涎液化糖链抗原-6(KL-6)在结缔组织病相关间质性肺病诊断中的应用价值。 方法: 纳入2016年1月至2017年12月就诊于新疆维吾尔自治区人民医院风湿免疫科并确诊的结缔组织病合并间质性肺病患者84例(CTD-ILD组),结缔组织病患者91例(CTD组)。采用酶联免疫吸附法测定血清KL-6水平。 结果: CTD-ILD组血清KL-6浓度明显高于CTD组[1 239(577,2 094))比152(89,280)U/ml](P<0.001)。KL-6诊断CTD-ILD最佳临界值为402 U/ml,敏感度、特异度分别为82.1%、86.8%,曲线下面积为0.905。Logistic回归分析结果显示,一氧化碳弥散量占预计值百分比(DLcoSB%)降低及KL-6水平升高与CTD-ILD发生独立相关,DLcoSB%降低(OR=0.928,95%CI:0.891~0.968)及KL-6水平升高(OR=1.005,95%CI:1.003~1.007)是CTD-ILD发生的独立危险因素。 结论: 血清KL-6是诊断CTD-ILD的重要血清学标志物,KL-6水平升高应警惕ILD的发生。.
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  • 文章类型: Journal Article
    Objective: To investigate the clinical significance of detection of myositis-specific antibodies (MSAs) and myositis-associated antibodies (MAAs) in patients with connective tissue disease-associated interstitial lung diseases (CTD-ILD). Methods: Serum samples of 120 patients with CTD-ILD admitted to the Department of Respiratory, Affiliated Drum Tower Hospital of Nanjing University Medical College from December 2016 to April 2018 were collected for analysis. The patients included 45 with polymyositis/dermatomyositis (PM/DM), 36 with Sjogren\'s syndrome (SS) and 39 with undifferentiated connective tissue disease (UCTD). There were 37 males and 83 females with an average age of (56±11) years. Thirty-two patients with non-CTD-ILD, 10 males and 22 females with an average age of (42±17) years, were enrolled as the control group. Euroline Autoimmune Inflammatory Myopathies 16 Ag kit was used for detecting MSAs and MAAs, and the positive rates of serum MSAs and MAAs were calculated. The antibody distribution and clinical characteristics of different groups were analyzed and compared. Results: Eighty-nine of the 120 patients with CTD-ILD were positive for MSA and/or MAA (74.2%), and the detection rates of MSAs and MAAs were 52.5% (63/120) and 61.7% (74/120) respectively. No myositis antibody was detected in the non-CTD-ILD group. The detection rates of MSAs in PM/DM-ILD group, SS-ILD group and UCTD-ILD group were 75.6% (34/45), 33.3%(12/36) and 43.6%(17/39) respectively. The total detection rate of MSAs in PM/DM group was significantly higher than that in SS group and UCTD group (χ(2)=14.53, 8.95, 0.01). The anti-ARS was the most frequent (50/120, 41.7%). The positive rates of MAAs in the three groups were 64.4%(29/45), 77.8%(28/36), 43.6%(17/39) respectively, and anti-Ro-52 accounted for 60%(72/120), and were highly correlated with MSAs such as anti-Jo-1 antibodies. Conclusion: Myositis antibody profiling should be performed in patients with ILD who were negative for conventional autoimmune antibody testing and had no CTD. In patients with SS-ILD and UCTD-ILD, the myositis antibody spectrum could detect the presence of myositis-specific antibodies and myositis-related antibodies in some patients, and its role in clinical diagnosis and treatment needed further observation.
    目的: 探讨结缔组织病相关间质性肺病(CTD-ILD)患者血清肌炎特异性抗体(MSA)与肌炎相关性抗体(MAA)检测的临床意义。 方法: 回顾性纳入2016年12月至2018年4月南京大学医学院附属鼓楼医院呼吸科就诊的120例住院CTD-ILD患者,其中男37例,女83例,年龄31~76岁,平均(56±11)岁。其中45例多发性肌炎/皮肌炎(PM/DM),36例干燥综合征(SS),39例未分化结缔组织病(UCTD)。纳入同期32名非CTD-ILD者为对照组,其中男10名,女22名,年龄21~79岁,平均(42±17)岁。选取保存的患者血清,采用欧蒙免疫印迹膜条方法进行MSA和MAA检测。计算血清MSA和MAA阳性率,分析比较各类CTD-ILD患者抗体分布情况及不同抗体血清阳性者的临床特征。 结果: 120例CTD-ILD患者中,89例MSA和(或)MAA阳性(74.2%),MSA和MAA检出率分别为52.5%(63/120)和61.7%(74/120);非CTD-ILD对照组未检测到肌炎抗体。其中PM/DM-ILD、SS-ILD及UCTD-ILD患者的MSA检出率分别为34/45、12/36、17/39,PM/DM-ILD患者的MSA总检出率明显高于SS-ILD和UCTD-ILD(χ(2)=14.53、8.95,P<0.01)。MSA中抗合成酶抗体阳性率最高(50/120,41.7%)。PM/DM-ILD、SS-ILD及UCTD-ILD患者MAA阳性率分别为29/45、28/36及17/39,抗RO-52抗体阳性率最高(72/120,60.0%),并且与MSA中抗Jo-1抗体等高度相关。 结论: 对于常规自身免疫抗体检测阴性无CTD的ILD患者应进行肌炎抗体谱检测。明确为SS-ILD、UCTD-ILD患者,肌炎抗体谱能检测部分患者有肌炎特异性抗体和肌炎相关性抗体存在,其对临床诊断和治疗的作用有待进一步观察。.
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  • 文章类型: Journal Article
    Connective tissue disease (CTD) is a collection of disorders characterized by various signs and symptoms such as circulation of autoantibodies in the entire system causing damage to internal organs. Interstitial lung disease (ILD) which is associated with CTD is referred to as CTD-ILD. Patients diagnosed with ILD should be thoroughly examined for the co-occurrence of CTD, since the treatment procedures and prognosis of CTD-ILD are vary from those of idiopathic interstitial pneumonia. The representative types of CTD which may accompany ILD include rheumatoid arthritis, systemic sclerosis (SSc), Sjögren\'s syndrome, mixed CTD, idiopathic inflammatory myopathies, and systemic lupus erythematous. Of these, ILD most frequently co-exists with SSc. If an ILD is observed in the chest, high resolution computed tomography and specific diagnostic criteria for any type of CTD are met, then a diagnosis of CTD-ILD is made. It is challenging to conduct a properly designed randomized study on CTD-ILD, due to low incidence. Therefore, CTD-ILD treatment approach is yet to been established in absence of randomized controlled clinical trials, with the exception of SSc-ILD. When a patient is presented with acute CTD-ILD or if symptoms occur due to progression of the disease, steroid and immunosuppressive therapy are generally considered.
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