Scleromyxedema: Response to high-dose intravenous immunoglobulin (hdIVIg)
Scleromyxedema: Response to high-dose intravenous immunoglobulin (hdIVIg)
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DOI：
10.1067/mjd.2000.104001
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发表时间：
2000-08-01
影响因子：
13.8
通讯作者：
Rustin, MHA
中科院分区：
文献类型：
--
作者：
Lister, RK;Jolles, S;Rustin, MHA
我们报告了2例硬化性黏液性水肿症患者，均伴有免疫球蛋白血症，每月给予大剂量静脉注射免疫球蛋白(HdIVIg)2g/kg。使用最初为硬皮病患者建立的客观皮肤评分系统评估治疗反应。该系统对硬结的总体严重程度和皮肤流动性的降低进行分级。两名患者最初都对一名患者的治疗产生了戏剧性的反应。第一名患者是一名30岁的黑人男子，在3个月的时间里，他的皮肤评分从36/60下降到11/60，在此期间他注射了3次hdIVIg。在意外中断治疗2个月后，出现了严重的神经肌肉并发症。最初，随着更频繁地注射hdIVIg，这些症状有所改善，但需要口服皮质类固醇来治疗恶化的肌病。不幸的是，对hdIVIg的初步反应没有持续，他1岁时的皮肤评分恢复到基线水平。第二名患者是一名60岁的白人男子，在只接受2次hdIVIg注射后，在3个月的时间里，皮肤评分从36/60下降到15/60，表现出类似的戏剧性下降。经过10个月的治疗后持续改善，hdIVIg输注间隔增加到10周而没有恶化。HdIVIg可能是一些硬化性粘液性水肿症的有效治疗方法，这是一种罕见的疾病，几乎没有有效的治疗方法，预后也很差。
We report 2 patients with scleromyxedema, both associated with IgG-lambda paraproteinemia, who were treated with high-dose intravenous immunoglobulin (hdIVIg) 2 g/kg per month. The response to treatment was assessed using an objective skin scoring system initially established for patients with scleroderma. This system grades the overall severity of the induration and the reduction in mobility of the skin. Both patients initially had a dramatic response to treatment which was sustained in one patient. The first patient, a 30-year-old black man, showed a reduction in skin scores from 36/60 to 11/60 over a 3-month period, during which time he had 3 infusions of hdIVIg. After an unplanned 2-month break from treatment, severe neuromuscular complications developed. These improved initially with more frequent infusions of hdIVIg but oral corticosteroids were required to treat worsening myopathy. Unfortunately the initial response to hdIVIg has not been sustained and his skin scores at 1 year returned to baseline. The second patient, a 60-year-old white man, showed a similarly dramatic reduction in skin scores from 36/60 to 15/60 over a 3-month period after having received only 2 infusions of hdIVIg. There has been sustained improvement after 10 months of therapy and the interval between hdIVIg infusions has been increased to 10 weeks without deterioration. HdIVIg may be an effective treatment for some patients with scleromyxedema, a rare condition with few effective treatments and a poor prognosis.