Goodpasture症候群における血漿交換療法

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Although the introduction of plasma exchange to the treatment of Goodpasture's syndrome has ameliorated pulmonary symptoms and markedly improved the prognosis of patients, the improvement in renal function has been unsatisfactory and many patients require maintenance hemodialysis. In addition, some patients still clie of respiratory failure and infection under treatment, so the outcome of the disease in these patients remains poor. In this article, we reviewed 19 patients with Goodpasture's syndrome treated with plasmapheresis during the 16-year period between 1982 and 1997 in Japan. We analyzed the relationship between the prognosis and the period from hospitalisation to initiation of plasmapheresis, the treatment method used, and the number of the sessions performed. Plasmapheresis was performed an average of 5.5 times within 2 weeks of starting treatment, and was performed a total of 6.3 times on average. A mean volume of 2.4 L of replacement fluid was used per procedure. Of the 12 surviving patients. 5 were able to cease dialysis, but the remaining 7 required maintenance dialysis. Of the 7 deaths, 3 were from infection and 4 were from respiratory failure. A comparison with European and American reports indicated that plasmapheresis was done less frequently and with a smaller volume of replacement fluid in Japan. However, the treatment was considered unlikely to produce any improvement in renal function in the majority of patients under hemodialysis at the initiation of combination treatment, since these patients had already developed end-stage renal failure and histologic examination also showed irreversible glomerular damage.
日本アフェレシス学会の論文
1998-05-31

Goodpasture症候群における血漿交換療法

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