Endothelial dysfunction precedes C-fiber abnormalities in primary (AL) amyloidosis.

摘要: Primary (AL; immunoglobulin light-chain associated) amyloidosis is characterized by the deposition of pathological proteins in extracellular matrix tissues and organs. Autonomic sensory peripheral neuropathy a common feature this disorder. The pathogenesis poorly defined. aims study were to investigate vascular neural function cutaneous microcirculation AL patients. Seven patients with controls studied. Acetylcholine sodium nitroprusside iontophoresed into forearm skin. Endothelial, smooth muscle, C-fiber-mediated blood flow (CuBF) recorded laser Doppler flowmetry. Endothelial vasodilation skin was attenuated (p = 0.007). Maximum endothelium-mediated CuBF patient group reduced 0.047). No differences could be detected C-fiber response or muscle value not significant). endothelium-independent did differ between two groups Early disease, present impaired endothelial function. At stage, are still preserved. These data suggest that abnormalities precede may contribute associated amyloidosis.