Harris Edward S, Meiselman Herbert J, Moriarty Patrick M, Metzger Allan, Malkovsky Miroslav
Department of Medicine, University of Wisconsin, Madison, WI, USA.
Department of Physiology & Biophysics, Keck School of Medicine, University of Southern California, Los Angeles, CA, USA.
J Scleroderma Relat Disord. 2018 Jun;3(2):132-152. doi: 10.1177/2397198318758606. Epub 2018 Mar 9.
Therapeutic plasma exchange has been tried as a treatment approach for systemic sclerosis since 1978 based on the rationale that some circulating factor is involved in disease pathogenesis, for example, autoantibodies or immune complexes, and that removing the potential pathogenic factors could lead to symptom improvement. Based on our impression that clinicians and researchers are largely unaware that a large volume of research has been published about the use of therapeutic plasma exchange as a treatment for systemic sclerosis, we conducted a comprehensive review and analysis of all published research on this topic.
We identified 46 relevant articles that met our search criteria, involving a total of 572 patients. Of these, 19 were case studies; the rest ranged from small observational studies to prospective randomized clinical trials. In all but two studies, most patients receiving therapeutic plasma exchange showed improvements in both clinical symptoms and laboratory markers, including significant improvement in Raynaud's symptoms and healing of digital ulceration after three to four weekly treatments. The beneficial effects from even a short course of therapeutic plasma exchange treatments were long-lasting, typically 6 months or longer. Therapeutic plasma exchange was very well tolerated. Adverse events were rare and, in almost all cases, mild and transitory.
These results suggest that long-term therapeutic plasma exchange may offer a low-risk way to control and in some cases reverse systemic sclerosis symptoms. The mechanism for the clinical improvements seen from therapeutic plasma exchange in systemic sclerosis patients is unclear. Therefore, additional studies of therapeutic plasma exchange effects in systemic sclerosis appear to be highly desirable.
自1978年以来，治疗性血浆置换就被尝试作为系统性硬化症的一种治疗方法，其依据是某些循环因子参与疾病发病机制，例如自身抗体或免疫复合物，并且去除潜在的致病因素可能会导致症状改善。基于我们的印象，临床医生和研究人员很大程度上并未意识到已经发表了大量关于使用治疗性血浆置换治疗系统性硬化症的研究，我们对关于该主题的所有已发表研究进行了全面回顾和分析。
我们确定了46篇符合我们搜索标准的相关文章，共涉及572名患者。其中，19篇为病例研究；其余的范围从小型观察性研究到前瞻性随机临床试验。除两项研究外，在所有研究中，大多数接受治疗性血浆置换的患者临床症状和实验室指标均有改善，包括雷诺氏症状显著改善以及经过三到四周每周一次的治疗后指端溃疡愈合。即使是短期的治疗性血浆置换治疗所产生的有益效果也是持久的，通常为6个月或更长时间。治疗性血浆置换耐受性良好。不良事件很少见，而且在几乎所有情况下都很轻微且短暂。
这些结果表明，长期治疗性血浆置换可能提供一种低风险的方法来控制并在某些情况下逆转系统性硬化症症状。系统性硬化症患者通过治疗性血浆置换实现临床改善的机制尚不清楚。因此，对系统性硬化症中治疗性血浆置换效果进行更多研究似乎非常必要。
