进行性系统性硬化症临床变化的测量：N-乙酰半胱氨酸的1年双盲安慰剂对照试验。
Measurement of clinical change in progressive systemic sclerosis: a 1 year double-blind placebo-controlled trial of N-acetylcysteine.
作者信息
Furst D E, Clements P J, Harris R, Ross M, Levy J, Paulus H E
出版信息
Ann Rheum Dis. 1979 Aug;38(4):356-61. doi: 10.1136/ard.38.4.356.
Abstract
N-acetylcysteine was employed as a therapeutic agent in a 1-year, parallel, double-blind, placebo-controlled, prospective study of 22 patients with progressive systemic sclerosis (PSS). Patients were closely followed-up and were extensively evaluated at baseline, 6 and 12 months. Most parameters remained unchanged during 1 year of study in both the NAC and placebo groups, which showed the need for prolonged studies of drugs in PSS if definitive results are to be obtained. Over the year of the study the diffusing capacity decreased in both groups, while several other parameters measured both increased and decreased. Several simple measures of function and skin distensibility were tested and two (oral aperature and handspread) were reproducible and sensitive enough to measure moderate changes of disease activity. They may be useful in future trials of drug effect in PSS.
摘要
在一项针对22例进行性系统性硬化症（PSS）患者的为期1年的平行、双盲、安慰剂对照前瞻性研究中，使用N-乙酰半胱氨酸作为治疗药物。对患者进行密切随访，并在基线、6个月和12个月时进行全面评估。在NAC组和安慰剂组的1年研究期间，大多数参数保持不变，这表明如果要获得明确结果，需要对PSS药物进行长期研究。在研究的这一年中，两组的弥散能力均下降，而其他几个测量参数则有升有降。测试了几种简单的功能和皮肤伸展性测量方法，其中两种（口腔开口度和手部伸展度）可重复且足够敏感，能够测量疾病活动的中度变化。它们可能在未来PSS药物疗效试验中有用。
相似文献
1
Measurement of clinical change in progressive systemic sclerosis: a 1 year double-blind placebo-controlled trial of N-acetylcysteine.进行性系统性硬化症临床变化的测量：N-乙酰半胱氨酸的1年双盲安慰剂对照试验。
Ann Rheum Dis. 1979 Aug;38(4):356-61. doi: 10.1136/ard.38.4.356.
2
Effects of N-acetylcysteine on Pulmonary Functions in Patients with Systemic Sclerosis: A Randomized Double Blind, Placebo Controlled Study.N-乙酰半胱氨酸对系统性硬化症患者肺功能的影响：一项随机、双盲、安慰剂对照研究。
Curr Rheumatol Rev. 2020;16(2):149-157. doi: 10.2174/1573397115666191212092608.
3
4
Longitudinal changes in lung function and respiratory symptoms in progressive systemic sclerosis. Prospective study.
Am J Med. 1987 Jul;83(1):83-92. doi: 10.1016/0002-9343(87)90501-8.
5
Randomized, prospective, placebo-controlled trial of bosentan in interstitial lung disease secondary to systemic sclerosis.波生坦治疗系统性硬化症继发间质性肺病的随机、前瞻性、安慰剂对照试验
Arthritis Rheum. 2010 Jul;62(7):2101-8. doi: 10.1002/art.27466.
6
Effect of mycophenolate mofetil (MMF) on systemic sclerosis-related interstitial lung disease with mildly impaired lung function: a double-blind, placebo-controlled, randomized trial.霉酚酸酯（MMF）治疗肺功能轻度受损的系统性硬化症相关间质性肺病的疗效：一项双盲、安慰剂对照、随机试验。
Rheumatol Int. 2020 Feb;40(2):207-216. doi: 10.1007/s00296-019-04481-8. Epub 2019 Dec 7.
7
[Oral N-acetylcysteine in the treatment of Raynaud's phenomenon secondary to systemic sclerosis: a randomized, double-blind, placebo-controlled clinical trial].口服N-乙酰半胱氨酸治疗系统性硬化症继发雷诺现象：一项随机、双盲、安慰剂对照临床试验
Rev Bras Reumatol. 2014 Nov-Dec;54(6):452-8. doi: 10.1016/j.rbr.2014.07.001. Epub 2014 Aug 27.
8
A multicenter, prospective, randomized, double-blind, placebo-controlled trial of corticosteroids and intravenous cyclophosphamide followed by oral azathioprine for the treatment of pulmonary fibrosis in scleroderma.一项多中心、前瞻性、随机、双盲、安慰剂对照试验，研究皮质类固醇和静脉注射环磷酰胺随后口服硫唑嘌呤治疗硬皮病相关性肺纤维化的疗效。
Arthritis Rheum. 2006 Dec;54(12):3962-70. doi: 10.1002/art.22204.
9
Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial.托西珠单抗治疗系统性硬化症：一项随机、双盲、安慰剂对照、3 期临床试验。
Lancet Respir Med. 2020 Oct;8(10):963-974. doi: 10.1016/S2213-2600(20)30318-0. Epub 2020 Aug 28.
10
A study protocol for the N-ICE trial: A randomised double-blind placebo-controlled study of the safety and efficacy of N-acetyl-cysteine (NAC) as a pharmacotherapy for methamphetamine ("ice") dependence.N-ICE 试验方案：乙酰半胱氨酸（NAC）作为治疗甲基苯丙胺（“冰毒”）依赖的药物治疗的安全性和有效性的随机、双盲、安慰剂对照研究。
Trials. 2019 Jun 4;20(1):325. doi: 10.1186/s13063-019-3450-0.
引用本文的文献
1
A review of recent studies on the pathogenesis of Systemic Sclerosis: focus on fibrosis pathways.系统性硬化症发病机制的近期研究综述：聚焦纤维化途径
Front Immunol. 2025 Apr 16;16:1551911. doi: 10.3389/fimmu.2025.1551911. eCollection 2025.
2
The challenges and controversies of measuring disease activity in systemic sclerosis.系统性硬化症疾病活动度测量的挑战与争议
J Scleroderma Relat Disord. 2018 Jun;3(2):115-121. doi: 10.1177/2397198318765061. Epub 2018 Mar 27.
3
Oxidative Stress Induced by Reactive Oxygen Species (ROS) and NADPH Oxidase 4 (NOX4) in the Pathogenesis of the Fibrotic Process in Systemic Sclerosis: A Promising Therapeutic Target.活性氧（ROS）和NADPH氧化酶4（NOX4）诱导的氧化应激在系统性硬化症纤维化过程发病机制中的作用：一个有前景的治疗靶点
J Clin Med. 2021 Oct 19;10(20):4791. doi: 10.3390/jcm10204791.
4
Role of cellular senescence and NOX4-mediated oxidative stress in systemic sclerosis pathogenesis.细胞衰老和NOX4介导的氧化应激在系统性硬化症发病机制中的作用。
Curr Rheumatol Rep. 2015 Jan;17(1):473. doi: 10.1007/s11926-014-0473-0.
5
New insights into the role of oxidative stress in scleroderma fibrosis.氧化应激在硬皮病纤维化中作用的新见解。
Open Rheumatol J. 2012;6:87-95. doi: 10.2174/1874312901206010087. Epub 2012 Jun 15.
6
Patterns and predictors of change in outcome measures in clinical trials in scleroderma: an individual patient meta-analysis of 629 subjects with diffuse cutaneous systemic sclerosis.硬皮病临床试验中结局指标变化的模式与预测因素：对629例弥漫性皮肤型系统性硬化症患者的个体患者荟萃分析
Arthritis Rheum. 2012 Oct;64(10):3420-9. doi: 10.1002/art.34427.
7
Dexamethasone pulse therapy in patients of systemic sclerosis: is it a viable proposition? A study from kashmir.全身硬化症患者的地塞米松脉冲疗法：这是一个可行的方案吗？来自克什米尔的一项研究。
Indian J Dermatol. 2010 Oct;55(4):355-8. doi: 10.4103/0019-5154.74543.
8
Reliability and validity of the delta finger-to-palm (FTP), a new measure of finger range of motion in systemic sclerosis.德尔塔指掌距（FTP）作为一种新的系统性硬化症手指活动范围的测量方法，其可靠性和有效性。
Clin Exp Rheumatol. 2010 Mar-Apr;28(2 Suppl 58):S28-36. Epub 2010 Jun 10.
9
Progressive systemic sclerosis and S-adenosylmethionine.进行性系统性硬化症与S-腺苷甲硫氨酸
Clin Rheumatol. 1985 Sep;4(3):360-1. doi: 10.1007/BF02031624.
10
N-acetylcysteine added to saliva does not affect IgA concentration or the agglutination of Streptococcus mutans in vitro.添加到唾液中的N-乙酰半胱氨酸在体外不影响IgA浓度或变形链球菌的凝集。
Eur J Clin Pharmacol. 1988;34(2):201-5. doi: 10.1007/BF00614559.
本文引用的文献
1
AN EARLY PULMONARY PHYSIOLOGIC ABNORMALITY IN PROGRESSIVE SYSTEMIC SCLEROSIS (DIFFUSE SCLERODERMA).进行性系统性硬化症（弥漫性硬皮病）早期的肺生理异常。
Am J Med. 1964 Mar;36:361-9. doi: 10.1016/0002-9343(64)90162-7.
2
The reduction of experimentally induced inflammation by sulfhydryl compounds.巯基化合物对实验性诱导炎症的减轻作用。
Biochem Pharmacol. 1967 Jul 7;16(7):1175-82. doi: 10.1016/0006-2952(67)90149-9.
3
Pathologic observations in systemic sclerosis (scleroderma). A study of fifty-eight autopsy cases and fifty-eight matched controls.系统性硬化症（硬皮病）的病理学观察。对58例尸检病例和58例匹配对照的研究。
Am J Med. 1969 Mar;46(3):428-40. doi: 10.1016/0002-9343(69)90044-8.
4
Generalized scleroderma. Treatment with an immunosuppressive agent.全身性硬皮病。用免疫抑制剂治疗。
Arch Dermatol. 1968 Jun;97(6):690-8. doi: 10.1001/archderm.97.6.690.
5
Effect of thiols in biological systems on protein sulfhydryl content.生物系统中的硫醇对蛋白质巯基含量的影响。
Biochem Pharmacol. 1970 May;19(5):1551-60. doi: 10.1016/0006-2952(70)90143-7.
6
N-acetylcysteine inactivates migration inhibitory factor and delayed hypersensitivity reactions.N-乙酰半胱氨酸可使迁移抑制因子和迟发型超敏反应失活。
Nat New Biol. 1971 Jun 2;231(22):157-9. doi: 10.1038/newbio231157a0.
7
Vascular disease in progressive systemic sclerosis (scleroderma).
Ann Intern Med. 1970 Aug;73(2):317-24. doi: 10.7326/0003-4819-73-2-317.
8
An assessment of grip strength measurement in rheumatoid arthritis.类风湿关节炎握力测量的评估
Scand J Rheumatol. 1974;3(1):17-23. doi: 10.3109/03009747409165124.
9
Progressive systemic sclerosis (diffuse scleroderma). A follow-up report of treatment with potaba.进行性系统性硬化症（弥漫性硬皮病）。关于使用对氨基苯甲酸钾治疗的随访报告。
S Afr Med J. 1973 Jun 16;47(23):1001-2.
10
Sulfhydryl deficiency in connective tissue disorders: correlation with disease activity and protein alterations.
Metabolism. 1971 May;20(5):446-55. doi: 10.1016/0026-0495(71)90120-x.
Zotero 插件