更新于：2024-11-01

Interstitial lung disease due to connective tissue disease

结缔组织病相关间质性肺疾病

更新于：2024-11-01

基本信息

别名

Interstitial lung disease due to connective tissue disease、Interstitial lung disease due to connective tissue disease (disorder)、interstitial lung disease due to connective tissue disease

+ [1]

简介-

关联

项与结缔组织病相关间质性肺疾病相关的药物

Belimumab

贝利尤单抗

靶点

BAFF

作用机制

BAFF抑制剂

在研机构

原研机构

在研适应症

非在研适应症

最高研发阶段批准上市

首次获批国家/地区

美国

首次获批日期2011-03-09

Genakumab

金纳单抗

靶点

IL-1β

作用机制

IL-1β抑制剂

在研机构

长春金赛药业有限责任公司

原研机构

长春金赛药业有限责任公司

在研适应症

痛风性关节炎 [+6]

非在研适应症-

最高研发阶段申请上市

首次获批国家/地区-

首次获批日期1800-01-20

项与结缔组织病相关间质性肺疾病相关的临床试验

NCT06644144 / Not yet recruitingN/AIIT

Early Identification of Progressive Pulmonary Fibrosis, Precision Medicine for More Oxygen - ILD Extension.

The goal of this observational study is to identify early biomarkers that can predict the development of progressive pulmonary fibrosis (PPF) in participants with interstitial lung diseases (ILDs). The participant population includes adults diagnosed with idiopathic pulmonary fibrosis (IPF), familial pulmonary fibrosis (FPF), other fibrotic ILDs, and interstitial lung abnormalities (ILA).
The main questions it aims to answer are:
* What biomarkers and risk factors are linked to fibrosis progression or can predict rapid worsening and sudden flare-ups in IPF and FPF patients?
* What biomarkers and risk factors can predict the development of a PPF phenotype in different types of ILD?
* What biomarkers and risk factors can help identify ILA patients who may develop significant ILD?
* What biomarkers and risk factors can predict how well ILD patients will respond to treatment?
Researchers will compare the outcomes between participants diagnosed with IPF/FPF, other fibrotic ILDs, and ILA to see if early detection biomarkers differ among these groups.
Participants will:
* Undergo blood sampling.
* Perform lung function tests.
* Have CT scans.
* Perform breath analysis
* Participate in exposome and microbiome analyses.
* Complete questionnaires.
* A subgroup of participants will be offered bronchoscopy.

开始日期2024-10-15

申办/合作机构

Stichting Onderzoek Cancer Center Amsterdam

[+2]

NCT06574581 / Recruiting临床1/2期IIT

Allogenic Adipose Tissue Derived-MSCs Therapy in Patients With Connective Tissue Diseases-associated Interstitial Lung Disease(CTD-ILD): Phase I/IIa Clinical Trial

Connective tissue disease (CTD), an autoimmune and inflammatory disease, usually accompanied by lung interstitial/alveolar inflammation and fibrosis (so called interstitial lung disease, ILD). The prevalence and mortality rate of CTD-ILD increase in recent several years. Although the use of corticosteroids and strong immunosuppressants can improve ILD in some patients with CTD, progressive lung fibrosis which needs lung transplantation and results in respiratory failure, even with mortality is observed. Currently, stem cell therapy is a breakthrough in the treatment of CTD-ILD, and the effective therapy with stem cells for patients with ILD have been reported.

开始日期2024-08-20

申办/合作机构

中国医药大学附设医院

ChiCTR2400080909 / SuspendedIIT

The study of ultrasound in screening and evaluating connective tissue disease associated interstitial lung disease and the construction of artificial intelligence evaluation model

开始日期2024-03-01

申办/合作机构-

100 项与结缔组织病相关间质性肺疾病相关的临床结果

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100 项与结缔组织病相关间质性肺疾病相关的转化医学

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0 项与结缔组织病相关间质性肺疾病相关的专利（医药）

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223

项与结缔组织病相关间质性肺疾病相关的文献（医药）

2024-10-18·International Journal of Ophthalmology

Microvascular alterations of the ocular surface and retina in connective tissue disease-related interstitial lung disease

Article

作者: Chen, Cheng ; Wei, Hong ; He, Liang-Qi ; Kang, Min ; Wu, Rui ; Huang, Hui ; Huang, Hui ; Ling, Qian ; Wang, Yi-Xin ; He, Liang-Qi ; Chen, Xu ; Zou, Jie ; Ling, Qian ; Xu, San-Hua ; Ying, Ping ; Xu, San-Hua ; Wang, Jun-Yi ; Ying, Ping ; Wang, Jun-Yi ; Chen, Li-Ming ; Shao, Yi ; Shao, Yi ; Chen, Xu ; Chen, Cheng ; Wei, Hong ; Wu, Rui ; Zou, Jie ; Wang, Yi-Xin

AIM: To examine the disparities in macular retinal vascular density between individuals with connective tissue disease-related interstitial lung disease (CTD-ILD) and healthy controls (HCs) by optical coherence tomography angiography (OCTA) and to investigate the changes in microvascular density in abnormal eyes. METHODS: For a retrospective case-control study, a total of 16 patients (32 eyes) diagnosed with CTD-ILD were selected as the ILD group. The 16 healthy volunteers with 32 eyes, matched in terms of age and sex with the patients, were recruited as control group. The macular retina’s superficial retinal layer (SRL) and deep retinal layer (DRL) were examined and scanned using OCTA in each individual eye. The densities of retinal microvascular (MIR), macrovascular (MAR), and total microvascular (TMI) were calculated and compared. Changes in retinal vascular density in the macular region were analyzed using three different segmentation methods: central annuli segmentation method (C1-C6), hemispheric segmentation method [uperior right (SR), superior left (SL), inferior left (IL), and inferior right (IR)], and Early Treatment Diabetic Retinopathy Study (ETDRS) methods [superior (S), inferior (I), left (L), and right (R)]. The data were analyzed using Version 9.0 of GraphPad prism and Pearson analysis. RESULTS: The OCTA data demonstrated a statistically significant difference (P<0.05) in macular retinal microvessel density between the two groups. Specifically, in the SRL and DRL analyses, the ILD group exhibited significantly lower surface density of MIR and TMI compared to the HCs group (P<0.05). Furthermore, using the hemispheric segmentation method, the ILD group showed notable reductions in SL, SR, and IL in the superficial retina (P<0.05), as well as marked decreases in SL and IR in the deep retina (P<0.05). Similarly, when employing the ETDRS method, the ILD group displayed substantial drops in superficial retinal S and I (P<0.05), along with notable reductions in deep retinal L, I, and R (P<0.05). In the central annuli segmentation method, the ILD group exhibited a significant decrease in the superficial retinal C2-4 region (P<0.05), whereas the deep retina showed a notable reduction in the C3-5 region (P<0.05). Additionally, there was an observed higher positive likelihood ratio in the superficial SR region and deep MIR. Furthermore, there was a negative correlation between conjunctival vascular density and both deep and superficial retinal TMI (P<0.001). CONCLUSION: Patients with CTD-ILD exhibits a significantly higher conjunctival vascular density compared to the HCs group. Conversely, their fundus retinal microvascular density is significantly lower. Furthermore, CTD-ILD patients display notably lower superficial and deep retinal vascular density in comparison to the HCs group. The inverse correlation between conjunctival vascular density and both superficial and deep retinal TMI suggests that detecting subtle changes in ocular microcirculation could potentially serve as an early diagnostic indicator for connective tissue diseases, thereby enhancing disease management.

2024-10-01·Autoimmunity Reviews

Corrigendum to “Practical guidance for the early recognition and follow-up of patients with connective tissue disease-related interstitial lung disease” [Autoimmunity Reviews - Volume 23, Issue 6, June 2024, 103582]

作者: De Vries-Bouwstra, Jeska K ; Søndergaard, Klaus ; Cordeiro, Ana ; De Vries-Bouwstra, Jeska K. ; Smith, Vanessa ; Tzouvelekis, Argyrios ; Guiot, Julien ; Dimitroulas, Theodoros ; Miedema, Jelle

2024-10-01·Autoimmunity Reviews

Cardiovascular disease in connective tissue disease-associated interstitial lung disease: A systematic review and meta-analysis of observational studies

Review

作者: Luo, Wenxuan ; Luo, Xiongyan ; Wang, Haolan ; Yang, Guanhui ; Huang, Jinyu ; Zhong, Jiaxun ; Wei, Xin ; Xiong, Anji ; Zheng, Xiaoli ; Hu, Ziyi

OBJECTIVESWe performed a systematic review and meta-analysis to assess whether patients with connective tissue disease (CTD)-associated interstitial lung diseases (ILD) have an increased prevalence of cardiovascular (CV) disease and to validate associated risk factors.METHODSThe PRISMA guidelines and PICO model were followed. We searched PubMed, Embase, Cochrane Library databases, Scopus, and Directory of Open Access Journals from inception to April 2024.RESULTSThirteen studies comprising of 12,520 patients were included. Patients with CTD-ILD had a significantly increased risk of CV disease than patients with CTD (relative risk [RR] = 1.65, 95 % confidence interval [CI]: 1.41, 1.93), which are related to the proportion of men (P = 0.001) and the proportion of smokers (P = 0.045). Subgroup analysis found that patients with CTD-ILD had a higher risk of heart failure (RR = 2.84, 95 % CI: 1.50, 5.39), arrhythmia (RR = 1.55, 95 % CI: 1.22, 1.97) than patients with CTD. Another subgroup analysis showed that RA-ILD and SSc-ILD were associated with an increased risk of CV disease, but not IIM-ILD and MCTD-ILD (RA-ILD: RR = 2.19, 95 % CI: 1.27, 3.80; SSc-ILD: RR = 1.53, 95 % CI: 1.29, 1.82). Besides, patients with CTD-ILD had a higher prevalence of pulmonary arterial hypertension (RR = 2.48, 95 % CI: 1.69, 3.63) than patients with CTD.CONCLUSIONSPatients with CTD-ILD had a 1.65 times increased risk of CV than patients with CTD-non-ILD, with increased prevalence of heart failure and arrhythmia. The risk of CV disease in SSc-ILD and RA-ILD is increased and we should pay more attention to male smokers. In addition, compared with CTD patients, CTD-ILD patients had a higher risk of pulmonary arterial hypertension.

项与结缔组织病相关间质性肺疾病相关的新闻（医药）

2024-08-25

·生物制品圈

GSK皮下注射「贝利尤单抗」在中国再获批临床

中国药品监督管理局药品审评中心（CDE）在其官方网站上最近发布了一项新的公告，宣布葛兰素史克公司（GSK）提交的贝利尤单抗注射液（皮下注射）获得了一项新的临床试验默示许可。这项新许可的目的是探索该药物在治疗成人结缔组织病相关间质性肺疾病（CTD-ILD）方面的潜力。贝利尤单抗是由GSK研发的靶向B淋巴细胞刺激因子（BLyS）的单克隆抗体。此前，贝利尤单抗的静脉注射剂型在中国已经获得了多项适应症的批准，这标志着其在治疗自身免疫性疾病方面的广泛应用。截图来源：CDE官网贝利尤单抗（商品名：belimumab）是一种人源化单克隆抗体，其作用机制是特异性结合B淋巴细胞刺激因子（BLyS），从而抑制B细胞的存活和增殖。这种药物能够减少自身免疫反应中异常活跃的B细胞数量，阻止它们分化成产生免疫球蛋白的浆细胞。 2019年7月，贝利尤单抗的静脉注射剂型在中国获得批准上市。目前，该药物在中国已被批准用于治疗成人和儿童的系统性红斑狼疮，以及与标准治疗联合使用，针对活动性狼疮肾炎（LN）的成年患者。与静脉注射相比，贝利尤单抗的皮下注射剂型具有缩短治疗时间和提高治疗便利性的潜力。根据中国药物临床试验与信息公示平台的官方信息，贝利尤单抗的皮下注射液在中国已经开展了两项临床研究。这包括一项针对中国系统性红斑狼疮（SLE）儿童的1期药代动力学研究，以及一项针对系统性硬化症相关间质性肺疾病（SSc-ILD）成人患者的国际多中心（含中国）2/3期有效性和安全性研究。在这项2/3期研究中，贝利尤单抗皮下注射液的给药方案是单次注射，每周一次，连续52周。结缔组织病相关间质性肺疾病（CTD-ILD）是一组与结缔组织疾病相关的肺部病变，这类病变在呼吸科中属于常见的纤维化性间质性肺病。当多种结缔组织疾病影响到肺间质时，可能会引起弥漫性肺泡和毛细血管功能单位的丧失，CTD-ILD是这些疾病中较为严重的临床表现之一。贝利尤单抗注射液（皮下注射）获得的新临床试验默示许可，预示着该药物将很快进入针对CTD-ILD患者的临床研究阶段。这一进展为这一患者群体带来了新的治疗选择。参考资料： [1] 中国国家药监局药品审评中心官网. Retrieved Aug 23, 2024, from https://www.cde.org.cn/main/xxgk/listpage/da6efd086c099b7fc949121166f0130c [2]中国药物临床试验与信息公示平台官网.From http://www.chinadrugtrials.org.cn/index.html 识别微信二维码，添加生物制品圈小编，符合条件者即可加入生物制品微信群！请注明：姓名+研究方向！版权声明本公众号所有转载文章系出于传递更多信息之目的，且明确注明来源和作者，不希望被转载的媒体或个人可与我们联系(cbplib@163.com)，我们将立即进行删除处理。所有文章仅代表作者观点，不代表本站立场。

临床1期