University of Utah and Veterans Affair Medical Center, Salt Lake City, USA.
Clin Exp Rheumatol. 2013 Mar-Apr;31(2 Suppl 76):166-71. Epub 2013 Jul 23.
Diffuse systemic sclerosis carries a high morbidity and mortality. The Prospective Registry of Early Systemic Sclerosis (PRESS), a multicentre incident cohort study of patients with early diffuse cutaneous systemic sclerosis, has the goal of advancing the understanding of disease pathogenesis and identifying novel biomarkers. In this review, PRESS investigators discuss the evidence pertaining to the more commonly used treatments for early diffuse SSc skin disease including methotrexate, mycophenolate, cyclophosphamide, azathioprine, and intravenous immunoglobulin. This review highlights the unmet need for effective treatment in early diffuse SSc as well as its more rigorous study. Nonetheless, the PRESS investigators aim to decrease intra- and inter-institutional variability in prescribing in order to improve the understanding of the clinical course of early diffuse SSc skin disease.
弥漫性系统性硬化症发病率和死亡率高。前瞻性早期系统性硬化症登记研究（PRESS）是一项多中心的早期弥漫性皮肤系统性硬化症患者的发病队列研究，旨在深入了解疾病发病机制并确定新的生物标志物。在这篇综述中，PRESS 研究人员讨论了更常用于治疗早期弥漫性 SSc 皮肤疾病的药物，包括甲氨蝶呤、霉酚酸酯、环磷酰胺、硫唑嘌呤和静脉注射免疫球蛋白。这篇综述强调了早期弥漫性 SSc 有效治疗的迫切需求以及对其更严格的研究。尽管如此，PRESS 研究人员旨在减少处方的机构内和机构间的变异性，以更好地了解早期弥漫性 SSc 皮肤疾病的临床病程。
