Endothelial dysfunction precedes C-fiber abnormalities in primary (AL) amyloidosis
Endothelial dysfunction precedes C-fiber abnormalities in primary (AL) amyloidosis
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DOI：
10.1002/ana.10552
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发表时间：
2003-06-01
影响因子：
11.2
通讯作者：
Freeman, R
中科院分区：
文献类型：
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作者：
Berghoff, M;Kathpal, M;Freeman, R
原发性（AL；免疫球蛋白轻链相关）淀粉样变性的特征是病理蛋白沉积在组织和器官的细胞外基质中。自主神经和感觉周围神经病变是这种疾病的共同特征。神经病的发病机制尚不清楚。本研究的目的是调查 AL 淀粉样变性患者皮肤微循环的血管和神经功能。对 7 名 AL 淀粉样变性患者和对照进行了研究。将乙酰胆碱和硝普钠离子导入前臂皮肤。通过激光多普勒血流计记录内皮血流、平滑肌血流和 C 纤维介导的皮肤血流 (CuBF)。 AL 淀粉样变性患者前臂皮肤内皮血管舒张减弱（p = 0.007）。患者组中内皮介导的最大 CuBF 降低 (p = 0.047)。在 C 纤维反应或平滑肌血管舒张方面未检测到组间差异（p 值不显着）。两组之间的最大 C 纤维和内皮依赖性 CuBF 没有差异（p 值不显着）。在疾病早期，AL 淀粉样变性患者表现出内皮功能受损。在此阶段，C纤维和平滑肌功能仍然保留。这些数据表明，内皮异常先于 AL 淀粉样变性相关神经病的发病机制，并可能导致其发病。
Primary (AL; immunoglobulin light-chain associated) amyloidosis is characterized by the deposition of pathological proteins in the extracellular matrix of tissues and organs. Autonomic and sensory peripheral neuropathy is a common feature of this disorder. The pathogenesis of the neuropathy is poorly defined. The aims of this study were to investigate vascular and neural function in the cutaneous microcirculation of AL amyloidosis patients. Seven patients with AL amyloidosis and controls were studied. Acetylcholine and sodium nitroprusside were iontophoresed into the forearm skin. Endothelial, smooth muscle, and C-fiber-mediated cutaneous blood flow (CuBF) were recorded by laser Doppler flowmetry. Endothelial vasodilation in the forearm skin was attenuated in AL amyloidosis patients (p = 0.007). Maximum endothelium-mediated CuBF in the patient group was reduced (p = 0.047). No group differences could be detected in the C-fiber response or smooth muscle vasodilation (p value not significant). Maximum C-fiber and endothelium-independent CuBF did not differ between the two groups (p value not significant). Early in the disease, AL amyloidosis patients present with impaired endothelial function. At this stage, C-fiber and smooth muscle function are still preserved. These data suggest that endothelial abnormalities precede and may contribute to the pathogenesis of the neuropathy associated with AL amyloidosis.