Abstract：
BACKGROUND: Systemic sclerosis (SSc) is a heterogenous, multi-system autoimmune disease that causes progressive fibrosis of the skin and internal organs, resulting in high morbidity and mortality. Intravenous Immunoglobulin (IVIG) is a therapeutic option for SSc; however, reports of its efficacy have been variable, and its use across multiple organ manifestations of SSc has not been comprehensively reviewed.
OBJECTIVE: The aim of this study was to systematically assess the existing literature on the role of IVIG use across a range of SSc manifestations.
METHODS: Medline, Embase, Cochrane, Web of Science and Scopus were searched from 01/01/2003-15/04/2024 using terms related to SSc and IVIG. Included studies were English-language full texts, where ≥5 adults with SSc received IVIG, and where a reportable outcome was documented.
RESULTS: Of 418 potentially relevant records, 12 were included in this review, comprising 266 patients across one randomised control trial, two pilot studies, one open label study, seven retrospective studies and one case control study. Eighteen outcomes were documented across five different organ systems: cutaneous, respiratory, musculoskeletal, gastrointestinal, and other (clinical improvement and corticosteroid sparing benefit). Results showed a favourable effect of IVIG in reducing the extent of skin thickening, muscle and joint pain, gastrointestinal symptoms, steroid dosing and improving patient/physician reported quality of life. Whilst IVIG may appear to be less beneficial for respiratory disease, the stabilisation in pulmonary function tests and radiological features may be considered a positive outcome in itself. Limitations included a lack of high-quality studies, and the use of concomitant therapies in many studies, rendering the efficacy of IVIG alone difficult to ascertain.
CONCLUSIONS: IVIG showed benefit in treating some manifestations of SSc, however there was a lack of convincing evidence for the efficacy in others. The lack of high-quality data highlights the need for further well-designed clinical trials to confirm these findings and inform guidelines for IVIG use.
OBJECTIVE: The aim of this study was to systematically assess the existing literature on the role of IVIG use across a range of SSc manifestations.
METHODS: Medline, Embase, Cochrane, Web of Science and Scopus were searched from 01/01/2003-15/04/2024 using terms related to SSc and IVIG. Included studies were English-language full texts, where ≥5 adults with SSc received IVIG, and where a reportable outcome was documented.
RESULTS: Of 418 potentially relevant records, 12 were included in this review, comprising 266 patients across one randomised control trial, two pilot studies, one open label study, seven retrospective studies and one case control study. Eighteen outcomes were documented across five different organ systems: cutaneous, respiratory, musculoskeletal, gastrointestinal, and other (clinical improvement and corticosteroid sparing benefit). Results showed a favourable effect of IVIG in reducing the extent of skin thickening, muscle and joint pain, gastrointestinal symptoms, steroid dosing and improving patient/physician reported quality of life. Whilst IVIG may appear to be less beneficial for respiratory disease, the stabilisation in pulmonary function tests and radiological features may be considered a positive outcome in itself. Limitations included a lack of high-quality studies, and the use of concomitant therapies in many studies, rendering the efficacy of IVIG alone difficult to ascertain.
CONCLUSIONS: IVIG showed benefit in treating some manifestations of SSc, however there was a lack of convincing evidence for the efficacy in others. The lack of high-quality data highlights the need for further well-designed clinical trials to confirm these findings and inform guidelines for IVIG use.
摘要：
背景：系统性硬化症（SSc）是一种异质性，导致皮肤和内脏器官进行性纤维化的多系统自身免疫性疾病，导致高发病率和死亡率。静脉免疫球蛋白（IVIG）是SSc的治疗选择;然而，关于其功效的报道是可变的，其在SSc多器官表现中的应用尚未得到全面审查。
目的：本研究的目的是系统地评估关于IVIG在一系列SSc表现中的作用的现有文献。
方法：Medline，Embase,科克伦,使用与SSc和IVIG相关的术语从2003/01-15/04/2024搜索WebofScience和Scopus。纳入的研究包括英语全文，≥5名SSc成年人接受IVIG，记录了可报告的结果。
结果：在418条潜在相关记录中，这次审查包括12个，包括一项随机对照试验的266名患者，两项试点研究，一项开放标签研究，7项回顾性研究和1项病例对照研究。在五个不同的器官系统中记录了18个结果：皮肤，呼吸,肌肉骨骼，胃肠,和其他（临床改善和保留皮质类固醇的益处）。结果表明，IVIG在减少皮肤增厚程度方面具有良好的效果，肌肉和关节疼痛，胃肠道症状,类固醇剂量和改善患者/医师报告的生活质量.虽然IVIG似乎对呼吸系统疾病不太有益，肺功能检查和放射学特征的稳定本身可能被认为是阳性结果。局限性包括缺乏高质量的研究，以及在许多研究中使用伴随疗法，使得单独的IVIG的功效难以确定。
结论：IVIG在治疗SSc的某些表现方面显示出益处，然而，缺乏令人信服的证据证明在其他方面的疗效。缺乏高质量的数据凸显了需要进一步精心设计的临床试验来确认这些发现并为IVIG使用指南提供信息。
目的：本研究的目的是系统地评估关于IVIG在一系列SSc表现中的作用的现有文献。
方法：Medline，Embase,科克伦,使用与SSc和IVIG相关的术语从2003/01-15/04/2024搜索WebofScience和Scopus。纳入的研究包括英语全文，≥5名SSc成年人接受IVIG，记录了可报告的结果。
结果：在418条潜在相关记录中，这次审查包括12个，包括一项随机对照试验的266名患者，两项试点研究，一项开放标签研究，7项回顾性研究和1项病例对照研究。在五个不同的器官系统中记录了18个结果：皮肤，呼吸,肌肉骨骼，胃肠,和其他（临床改善和保留皮质类固醇的益处）。结果表明，IVIG在减少皮肤增厚程度方面具有良好的效果，肌肉和关节疼痛，胃肠道症状,类固醇剂量和改善患者/医师报告的生活质量.虽然IVIG似乎对呼吸系统疾病不太有益，肺功能检查和放射学特征的稳定本身可能被认为是阳性结果。局限性包括缺乏高质量的研究，以及在许多研究中使用伴随疗法，使得单独的IVIG的功效难以确定。
结论：IVIG在治疗SSc的某些表现方面显示出益处，然而，缺乏令人信服的证据证明在其他方面的疗效。缺乏高质量的数据凸显了需要进一步精心设计的临床试验来确认这些发现并为IVIG使用指南提供信息。
