Iorizzo Luciano J, Jorizzo Joseph L
Department of Medicine, SUNY Upstate Medical University, Syracuse, New York 13210, USA.
J Am Acad Dermatol. 2008 Jul;59(1):99-112. doi: 10.1016/j.jaad.2008.02.043. Epub 2008 Apr 18.
Dermatomyositis (DM) is an idiopathic inflammatory myopathy. The mainstay of treatment for DM is oral corticosteroids. However, the dose and length of treatment is debated. Adding to the confusion, there have been no randomized controlled studies comparing the use of various corticosteroid doses and taper rates, and no controlled long-term studies assessing the hypothesis that, unlike systemic lupus erythematous, patients with DM can often achieve long-term remission off therapy. This literature review supports an approach that prednisone should be started at about 1 mg/kg/d, which is then tapered slowly based on the response. As patients respond differently to prednisone, additional therapies may be necessary. When to initiate these therapies requires clinical judgment. In addition, as we learn more about the pathophysiology of DM, newer medications that target specific mechanisms in the immune response may help us better treat the disease. Evidence-based data with long-term follow-up will allow for selection of the best treatment to maximize long-term remission, not simply short-term lowering of the systemic corticosteroid dose.
皮肌炎(DM)是一种特发性炎性肌病。DM治疗的主要手段是口服糖皮质激素。然而,治疗的剂量和疗程存在争议。更令人困惑的是,尚无随机对照研究比较不同糖皮质激素剂量和减量速率的使用情况,也没有对照的长期研究评估如下假设:与系统性红斑狼疮不同,DM患者通常在停药后可实现长期缓解。这篇文献综述支持一种方法,即泼尼松应起始剂量约为1mg/(kg·d),然后根据反应缓慢减量。由于患者对泼尼松的反应不同,可能需要额外的治疗。何时开始这些治疗需要临床判断。此外,随着我们对DM病理生理学了解得更多,针对免疫反应中特定机制的新型药物可能有助于我们更好地治疗该疾病。长期随访的循证数据将有助于选择最佳治疗方法,以实现长期缓解的最大化,而不仅仅是短期降低全身糖皮质激素剂量。