Recent studies on oxidative stress have revealed that free radical injury
appears to be involved in either the amyloid formation process or in post-fibrillar
modification in several types of amyloidosis. Here, we report the role
of oxidative stress in the pathogenesis of dialysis-related amyloidosis(DRA)
and familial amyloidotic polyneuropathy(FAP), and propose radical scavenger
treatment for such amyloidosis. For patients under maintenance hemodialysis,
EC-SOD Arg213Gly was a risk factor for the progression of DRA, atherosclerosis,
and renal failure causing hemodialysis. In FAP patients who had EC-SOD
Arg213Gly, massive amyloid deposition which may be related to increased
oxidative stress in loco was found especially prominently around blood
vessels in the interstitial tissues. Histological and biochemical examinations
revealed that oxidative stress is deeply connected with amyloid formation
mechanisms in FAP. We started radical scavenger therapy, such as N-acetyl
cysteine, vitamin E and vitamin C in 20 Swedish FAP patients for 6 months.
Although no improvement was found in the amount of amyloid deposition in
biopsy specimens, modified body mass index(mBMI), an index of nutritional
status, tended to be increased, suggesting the therapeutic possibility
of radical scavenger treatment for amyloidosis.
[Rinsho Byori 51 : 140〜145, 2003]
*1Department of Laboratory Medicine, Kumamoto University School of Medicine, Kumamoto 860-0811
【Key Words】amyloidosis(アミロイドーシス)，familial amyloidotic polyneuropathy(家族性アミロイドポリニューロパチー)，dialysis related amyloidosis(透析アミロイドーシス)，oxidative stress(酸化ストレス)，extracellular SOD：EC-SOD
*1,2熊本大学医学部臨床検査医学講座(〒860-0811 熊本市本荘1-1-1)
E-mail :m-nakamura@fu.kuh.kumamoto-u.ac.jp