Prolonged dimethylsulphoxide treatment in 13 patients with systemic amyloidosis.
Prolonged dimethylsulphoxide treatment in 13 patients with systemic amyloidosis.
复制标题
对 13 名系统性淀粉样变性患者进行长期二甲基亚砜治疗。
DOI：
10.1136/ard.41.6.587
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发表时间：
1982
影响因子：
27.4
通讯作者：
I. Kedar
中科院分区：
文献类型：
--
作者：
M. Ravid;J. Shapira;R. Lang;I. Kedar
对3例家族性地中海热（FMF）淀粉样变性患者、3例特发性淀粉样变性患者和7例继发性淀粉样变性患者给予持续口服二甲基亚砜（DMSO）治疗（7 ~ 15 g/d）。所有病例均以肾病综合征和不同程度肾功能不全为主要临床表现。以肾功能作为评价治疗效果的主要参数。DMSO治疗7-16个月对FMF患者和特发性淀粉样变性患者没有效果；他们都经历了可预测的临床病程，要么死于心力衰竭，要么一直在进行慢性血液透析。在7例继发性淀粉样变性患者中，在DMSO治疗3-6个月后，肾功能明显改善。结果显示肌酐清除率上升30-100%，蛋白尿下降。只要DMSO被使用，这种新的平衡就一直保持着。没有发现DMSO的严重副作用。轻度恶心和难闻的呼吸气味是患者的主要担忧。我们的结论是，口服DMSO治疗试验对所有继发性淀粉样变性患者都是必要的。这种治疗不愉快，但没有特别的风险。它可能显著延长寿命，尽管它对淀粉样蛋白沉积本身的影响值得怀疑。
Continuous oral dimethylsulphoxide (DMSO) treatment (7-15 g/day) was given to 3 patients with amyloidosis of familial Mediterranean fever (FMF), 3 patients with idiopathic amyloidosis, and 7 patients with secondary amyloidosis. The nephrotic syndrome and various degrees of renal insufficiency were the major clinical manifestation in all case. Renal function was used as the main parameter for evaluation of therapy. DMSO treatment for 7-16 months produced no effect in the FMF patients and in the patient with idiopathic amyloidosis; they all ran the predictable clinical course of their disease and either died of cardiac failure or have been maintained on chronic haemodialysis. In the 7 patients with secondary amyloidosis an unequivocal improvement of renal function was observed following 3-6 months of DMSO treatment. It was shown by a 30-100% rise of creatinine clearance and a decline in proteinuria. This new equilibrium has been maintained as long as DMSO was administered. No serious side effects of DMSO wee encountered. Mild nausea and an unpleasant breath odour were the patients' main concern. We conclude that a therapeutic trial with oral DMSO is warranted in all patients with secondary amyloidosis. This treatment is unpleasant but bears no exceptional risks. It may significantly prolong life, though its effect on amyloid deposits themselves is doubtful.