与自身免疫性疾病相关的进行性肺纤维化:文献系统回顾

Q3 Health Professions Revista Colombiana de Reumatologia Pub Date : 2024-04-01 DOI:10.1016/j.rcreu.2023.09.007
Luis Javier Cajas Santana , Diana Rocío Gil Calderón , Manuela Rubio Rivera , José Alex Yate , Javier Leonardo Galindo
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引用次数: 0

摘要

导言结缔组织病相关间质性肺病(CTD-ILD)占所有 ILD 病例的 30%。部分患者病情进展并发展为进行性肺纤维化,其预后与间质性肺纤维化相当。本研究回顾了有关流行病学、风险因素、生物标志物和治疗的相关证据。纳入了原始观察性和描述性文章。未提供 CTD 诊断信息的文章被排除在外。对 PUBMED、EMBASE、SCOPUS 和 LILACS 进行了检索。结果平均而言,这些患者的 ILD 随时间推移进展了 33.7%。进行性肺纤维化患者的 3.7 年中位生存期与特发性肺纤维化患者相似。死亡率明显增加(危险比 3.29;95% CI 2.76-3.82)。34%的硬皮病(SSc)相关ILD病例的病程呈进行性发展。六种干扰素诱导蛋白和由12种生物标志物组成的蛋白质组图谱用于预测病情进展和治疗反应。INBUILD和SENSCIS研究对宁替尼的疗效进行了评估,结果表明,宁替尼的强迫生命容量减少了。类风湿性关节炎(RA)相关 ILD 的进展率为 38% 至 50%。结论相当一部分 CTD-ILD 患者的肺部疾病会进展,并带来相应的不良死亡率影响。有关 CTD-ILD 的大部分数据来自 RA-ILD 和 SSc-ILD 患者组群,在这些组群中,抗纤维化药物和同时进行的免疫抑制治疗非常有效。有关其他自身免疫性疾病的资料尚不充分,因此无法就疾病进展率或治疗效果得出确切结论。
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Progressive pulmonary fibrosis associated autoimmune diseases: Systematic review of the literature

Introduction

Connective tissue disease-related interstitial lung disease (CTD-ILD) accounts for 30% of all cases of ILD. Some patients progress and develop progressive pulmonary fibrosis, which has a prognosis comparable to interstitial pulmonary fibrosis. In this study, relevant evidence about epidemiology, risk factors, biomarkers, and treatment are reviewed.

Materials and methods

A systematic review of the literature was carried out. Original observational and descriptive articles were included. Articles not providing information about the CTD diagnosis were excluded. PUBMED, EMBASE, SCOPUS, and LILACS were all searched. The total number of articles obtained was 528; data were extracted from 61 original articles.

Results

On average, ILD in these patients progressed by 33.7% over time. Patients with progressive pulmonary fibrosis had a similar 3.7-year median survival as those with idiopathic pulmonary fibrosis. Mortality was markedly increased (hazards ratio 3.29; 95% CI 2.76–3.82). A progressive course was seen in 34% of scleroderma (SSc) related ILD cases. Six interferon-induced proteins and a proteomic profile of 12 biomarkers were used to predict progression and response to treatment. The INBUILD and SENSCIS studies that assessed the effectiveness of nintedanib effectiveness revealed a reduced decrease in forced vital capacity. Progression in rheumatoid arthritis (RA) related ILD ranges from 38% to 50%. Control of RA disease activity and use of antifibrotics benefit the lungs.

Conclusion

A significant proportion of patients with CTD-ILD have progressive lung disease, with the corresponding adverse mortality effects. The majority of the data regarding CTD-ILD are from cohorts of patients with RA-ILD and SSc-ILD, in which antifibrotics and concurrent immunosuppressive treatments have been effective. There is not enough information available on other autoimmune disorders to draw any firm conclusions regarding progression rates or treatment effects.

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来源期刊
Revista Colombiana de Reumatologia
Revista Colombiana de Reumatologia Medicine-Rheumatology
CiteScore
0.80
自引率
0.00%
发文量
92
期刊介绍: The Colombian Journal of Rheumatology (Revista Colombiana de Reumatología) is the official organ of the Colombian Association of Rheumatology (Asociación Colombiana de Reumatología) and the Central American, Caribbean and Andean Association of Rheumatology (Asociación Centroamericana Caribe Andina de Reumatología) - ACCA. It was created in December 1993 with the purpose of disseminating scientific information derived from primary and secondary research and presenting cases coming from the practice of Rheumatology in Latin America. Since its foundation, the Journal has been characterized by its plurality with subjects of all rheumatic and osteomuscular pathologies, in the form of original articles, historical articles, economic evaluations, and articles of reflection and education in Medicine. It covers an extensive area of topics ranging from the broad spectrum of the clinical aspects of rheumatology and related areas in autoimmunity (both in pediatric and adult pathologies), to aspects of basic sciences. It is an academic tool for the different members of the academic and scientific community at their different levels of training, from undergraduate to post-doctoral degrees, managing to integrate all actors inter and trans disciplinarily. It is intended for rheumatologists, general internists, specialists in related areas, and general practitioners in the country and abroad. It has become an important space in the work of all rheumatologists from Central and South America.
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