曲前列尼尔
这一前列环素类似物已试验用于治疗雷诺现象和/或手指溃疡。[40]Engel G, Rockson SG. Treprostinil for the treatment of severe digital necrosis in systemic sclerosis. Vasc Med. 2005;10:29-32.http://vmj.sagepub.com/content/10/1/29.full.pdfhttp://www.ncbi.nlm.nih.gov/pubmed/15920997?tool=bestpractice.com[41]Chung L, Fiorentino D. A pilot trial of treprostinil for the treatment and prevention of digital ulcers in patients with systemic sclerosis. J Am Acad Dermatol. 2006;54:880-882.http://www.ncbi.nlm.nih.gov/pubmed/16635673?tool=bestpractice.com
靶抗体
已有针对肿瘤生长因子 (TGF) -β 和其他促纤维化信号的研究。[15]Gabrielli A, Avvedimento EV, Krieg T. Scleroderma. N Engl J Med. 2009 May 7;360(19):1989-2003.http://www.ncbi.nlm.nih.gov/pubmed/19420368?tool=bestpractice.com[42]Abraham DJ, Shiwen X, Black CM, et al. Tumor necrosis factor alpha suppresses the induction of connective tissue growth factor by transforming growth factor-beta in normal and scleroderma fibroblasts. J Biol Chem. 2000;275:15220-15225.http://www.jbc.org/content/275/20/15220.longhttp://www.ncbi.nlm.nih.gov/pubmed/10809757?tool=bestpractice.com 到目前为止,在随机对照试验中均无显示有效的。
利妥昔单抗
利妥昔单抗的初步研究表明其对皮肤和肺功能有所改进。[43]McGonagle D, Tan AL, Madden J, et al. Successful treatment of resistant scleroderma-associated interstitial lung disease with rituximab. Rheumatology (Oxford). 2008;47:552-553.http://www.ncbi.nlm.nih.gov/pubmed/18281368?tool=bestpractice.com[44]Daoussis D, Liossis SN, Tsamandas AC, et al. Experience with rituximab in scleroderma: results from a 1-year, proof-of-principle study. Rheumatology (Oxford). 2010;49:271-280.http://rheumatology.oxfordjournals.org/content/49/2/271.fullhttp://www.ncbi.nlm.nih.gov/pubmed/19447770?tool=bestpractice.com
伊马替尼
有人建议,使用酪氨酸激酶抑制剂(例如,伊马替尼)可改进系统性硬化症。[45]Papadakis V, Karakasis D, Sfikakis PP, et al. Is imatinib mesylate a promising drug in scleroderma due to extensive chronic graft-versus-host disease? Leuk Lymphoma. 2009;50:471-474.http://www.ncbi.nlm.nih.gov/pubmed/19253137?tool=bestpractice.com[46]Soria A, Cario-André M, Lepreux S, et al. The effect of imatinib (Glivec®) on scleroderma and normal dermal fibroblasts: a preclinical study. Dermatology. 2008;216:109-117.http://www.ncbi.nlm.nih.gov/pubmed/18216472?tool=bestpractice.com 然而,一项小型研究发现,在用于治疗活动性(多为早期)弥漫性皮肤系统性硬化症时,伊马替尼的耐受性差。[47]Pope J, McBain D, Petrlich L, et al. Imatinib in active diffuse cutaneous systemic sclerosis: Results of a six-month, randomized, double-blind, placebo-controlled, proof-of-concept pilot study at a single center. Arthritis Rheum. 2011;63:3547-3551.http://www.ncbi.nlm.nih.gov/pubmed/21769850?tool=bestpractice.com