Management of Widespread Skin Thickening in Diffuse Systemic Sclerosis.
Management of Widespread Skin Thickening in Diffuse Systemic Sclerosis.
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DOI：
10.1007/s40674-016-0040-0
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发表时间：
2016-03
影响因子：
1.2
通讯作者：
Clements P
中科院分区：
文献类型：
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作者：
Kafaja S;Clements P
皮肤增厚是系统性硬化症(SSC)的早期器官表现之一，对SSC患者的生活质量和日常生活质量有很大影响。随着疾病的进展和其他器官的进一步参与，发生的动态变化向治疗医生提出了治疗挑战。因此，当考虑皮肤病的药物治疗时，治疗医生应该考虑许多因素，包括疾病持续时间、皮肤增厚率、疾病进展程度、器官受累和患者相关的结果指标，所有这些因素都会影响所考虑的治疗类型。对于早期弥漫性皮肤病，我们倾向于使用甲氨蝶呤(MTX)。当有证据表明肺部受累或肌腱摩擦摩擦(鉴于其与ILD的发展相关)时，我们倾向于使用霉酚酸酯或环磷酰胺，因为这些药物已被证明对SSC肺部疾病的特定适应症有效。另一方面，我们已经控制了关节疾病，当存在MTX或其他DMARDS时，以及当有证据表明炎症性多发性关节炎或类风湿性关节炎重叠时，使用生物制剂。虽然SSc环境下的肌炎的治疗可能会出现治疗困境，但我们可以不情愿地与MTX、霉酚酸酯、静脉注射免疫球蛋白(IV-Ig)或利妥昔单抗一起使用类固醇。正在进行的临床试验研究tocilizumab、abatacept和其他药物的使用，提供了有希望的潜在治疗方法。SSC在治疗皮肤病方面取得了长足的进步。随着最近的试验将重点放在早期SSC疾病上，这将使人们能够更深入地了解SSC的潜在机制，特别是与皮肤有关的机制，并扩大该领域未来的治疗选择。
Skin thickening is one of the early organ manifestations of systemic sclerosis (SSc) and has a great impact on quality of life (QOL) as well as overall daily living in patients with SSc. The dynamic changes that occur as the disease progresses and as other organs become further involved present the treating physician with therapeutic challenges. Hence, when considering drug therapy for skin disease, the treating physician should consider a number of factors including disease duration, the rate of skin thickening, the extent of disease progression, organ involvements, and patient-related outcome measures, all of which impact the type of treatments considered. For early diffuse skin disease, we prefer the use of methotrexate (MTX). And when there is evidence of lung involvement or tendon friction rubs (given its association with ILD development), we tend to shift to the use of mycophenolate or cyclophosphamide because these agents have been shown efficacious for the specific indication of lung disease in SSc. We have managed joint disease, on the other hand when present, with MTX or other DMARDs, as well as the use of biologics when there is evidence of inflammatory polyarthritis or rheumatoid arthritis overlap. While the treatment of myositis in the setting of SSc can present a therapeutic dilemma, reluctantly, we may use steroids along with MTX, mycophenolate, intravenous immunoglobulin (IV-Ig), or rituximab. Ongoing clinical trials investigating the use of tocilizumab, abatacept, and other agents offer promising potential therapies. Great strides have been made in treating skin disease in SSc. And with recent trials focusing on early SSc disease, this will allow for a greater insight into the mechanisms underlying SSc especially as it relates to skin, and the expansion of future treatment options in this field.