Can we define difficult-to-treat systemic sclerosis?

IF 3.9 3区 医学 Q2 IMMUNOLOGY
Expert Review of Clinical Immunology Pub Date : 2024-09-01 Epub Date: 2024-05-14 DOI:10.1080/1744666X.2024.2352450
Gabriella Szűcs, Zoltán Szekanecz, Szilvia Szamosi
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引用次数: 0

Abstract

Introduction: Systemic sclerosis (SSc) is a chronic autoimmune rheumatic disease characterized by microvascular alterations, immunopathology, and widespread fibrosis involving various organs. It is considered difficult to treat due to several reasons: complex pathogenesis, heterogeneity, late diagnosis, limited treatment options for certain organ manifestations, lack of personalized medicine.

Areas covered: This review presents the heterogeneity, survival and organ manifestations with their risk factors of systemic sclerosis and their current treatment options, while drawing attention to difficult-to-treat forms of the disease, based on literature indexed in PubMed.

Expert opinion: Despite recent advances in the management of SSc over the last decades, the disease presents significant morbidity and mortality. Although available treatment protocols brought significant advancements in terms of survival in SSc-associated interstitial lung disease and pulmonary arterial hypertension, less success has been achieved in the treatment of Raynaud's phenomenon and digital ulcers and the results are modest in case of heart, gastrointestinal, and renal manifestations. There are patients who do not respond to treatment and deteriorate even with adequate therapy. They can be considered difficult-to treat (D2T) cases. We have created a possible score system based on the individual organ manifestations and highlighted treatment options for the D2T SSc category.

我们能否定义难以治疗的系统性硬化症?
导言:系统性硬化症(SSc)是一种慢性自身免疫性风湿病,以微血管改变、免疫病理和广泛纤维化为特征,累及多个器官。由于发病机制复杂、异质性强、诊断较晚、某些器官表现的治疗方案有限、缺乏个性化医疗等原因,该病被认为难以治疗:本综述以PubMed索引的文献为基础,介绍了系统性硬化症的异质性、存活率、器官表现及其风险因素,以及目前的治疗方案,同时提请注意该病的难治形式:尽管过去几十年来系统性硬化症的治疗取得了最新进展,但该病的发病率和死亡率仍然很高。虽然现有的治疗方案大大提高了SSc相关间质性肺病和肺动脉高压患者的存活率,但在治疗雷诺现象和数字溃疡方面却收效甚微,而在治疗心脏、胃肠道和肾脏表现方面也收效甚微。有些患者对治疗没有反应,即使接受了适当的治疗,病情也会恶化。这些患者可被视为难治病例(D2T)。我们根据各个器官的表现建立了一个可能的评分系统,并强调了D2T SSc类别的治疗方案。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
CiteScore
7.60
自引率
2.30%
发文量
221
审稿时长
6-12 weeks
期刊介绍: Expert Review of Clinical Immunology (ISSN 1744-666X) provides expert analysis and commentary regarding the performance of new therapeutic and diagnostic modalities in clinical immunology. Members of the International Editorial Advisory Panel of Expert Review of Clinical Immunology are the forefront of their area of expertise. This panel works with our dedicated editorial team to identify the most important and topical review themes and the corresponding expert(s) most appropriate to provide commentary and analysis. All articles are subject to rigorous peer-review, and the finished reviews provide an essential contribution to decision-making in clinical immunology. Articles focus on the following key areas: • Therapeutic overviews of specific immunologic disorders highlighting optimal therapy and prospects for new medicines • Performance and benefits of newly approved therapeutic agents • New diagnostic approaches • Screening and patient stratification • Pharmacoeconomic studies • New therapeutic indications for existing therapies • Adverse effects, occurrence and reduction • Prospects for medicines in late-stage trials approaching regulatory approval • Novel treatment strategies • Epidemiological studies • Commentary and comparison of treatment guidelines Topics include infection and immunity, inflammation, host defense mechanisms, congenital and acquired immunodeficiencies, anaphylaxis and allergy, systemic immune diseases, organ-specific inflammatory diseases, transplantation immunology, endocrinology and diabetes, cancer immunology, neuroimmunology and hematological diseases.
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