自身免疫性疾病肺动脉高压——筛查、诊断和治疗管理新概念综述

IF 0.1 Q4 MEDICINE, GENERAL & INTERNAL Romanian Journal of Military Medicine Pub Date : 2023-08-01 DOI:10.55453/rjmm.2023.126.4.13
D. Anghel, Iulia N. Nicolau, Liana I. Mihalca, O. Petrache, R. Costache, D. Opriș-Belinski, V. Bojinca, F. Ioniță Radu
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引用次数: 0

摘要

鉴于死亡率和发病率的增加,尤其是与自身免疫性疾病(PH的一个特定亚组)相关的肺动脉高压,肺动脉高压在过去几年中越来越受到关注,这些疾病在全球范围内的发病率和患病率都在增加。其中,系统性硬化症尤其令人感兴趣,是结缔组织疾病(CTDs)中最常见的,PH是主要并发症。其他CTD,如混合结缔组织病(MCTD)、系统性红斑狼疮(SLE)、原发性干燥综合征和皮肌炎,也可能发展为PH,作为这些严重复杂疾病的并发症,这可能对预后和生活质量产生重大影响。类风湿性关节炎和强直性脊柱炎也有PH的报道,但还需要进一步的研究。多项研究表明,系统性硬化症伴肺动脉高压(SSc-PH)患者的3年生存率在31%至52%之间,而5年生存率低于<50%。因此,在这些情况下,早期准确的诊断以及随后的PH管理是强制性的,因为尽管治疗取得了进展,但存活率仍然不理想。本文的目的是概述与PH相关的不同自身免疫性疾病,并提供当前最佳实践的简单参考来源。
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Pulmonary Hypertension in Autoimmune Diseases – A Review of New Concepts in Screening, Diagnosis and Therapeutic Management
Pulmonary hypertension (PH) has gained more and more attention in the last years given the increased mortality and morbidity rate and in particular pulmonary arterial hypertension associated with autoimmune diseases (a specific subgroup of PH), entities with an increasing incidence and prevalence globally. Among these, systemic sclerosis is of particular interest, being the most common among connective tissue diseases (CTDs) with PH as a major complication. Other CTDs such as mixed connective tissue disease (MCTD), systemic lupus erythematosus (SLE), primary Sjogren's syndrome, and dermatomyositis may also develop PH as a complication of these severe and complex conditions, which can have a major impact on prognosis and quality of life. PH has also been reported in rheumatoid arthritis and ankylosing spondylitis but further studies are necessary. Several studies have shown that the 3-year survival rate for patients with systemic sclerosis-associated with pulmonary hypertension (SSc-PH) is between 31% and 52%, while the 5-year survival rate is below <50%. Therefore, an early and accurate diagnosis as well as subsequent management of PH in these conditions is mandatory, since survival remains suboptimal despite treatment advances. The aim of this article is to provide an overview of different autoimmune diseases associated with PH and to provide an easy reference source on current best practices.
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来源期刊
Romanian Journal of Military Medicine
Romanian Journal of Military Medicine MEDICINE, GENERAL & INTERNAL-
自引率
33.30%
发文量
2
审稿时长
12 weeks
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