Endothelium-dependent vasodilatation in patients with familial amyloidoticpolyneuropathy

We examined endothelium-dependent vasodilatation in 15 familial amyloidotic polyneuropathy (FAP) amyloidogenic transthyretin (ATTR) Valine30Methionine (Val30Met) patients and 12 healthy volunteers. Using ultrasonography, we m easured the radial artery diameters under both baseline and hyperemic condi tions. Endothelium-dependent vasodilatation was expressed as a percent incr ease in the diameters of the radial artery after induced hyperemia. Endothe lium-dependent vasodilatation tended to decrease in the patients, compared with healthy volunteers. Responses were not elicited at all in patients wit h disease of more than 9 years' duration. Linear negative correlation was o bserved between endothelium-dependent vasodilatation and disease duration ( P < 0.01). Correlation between endothelium-dependent vasodilatation and deg ree of autonomic dysfunction was significant (P = 0.0524) and for age was c lose to significance (P = 0.051). These results suggest that the peripheral vasomotor dysfunction in FAP patients may predominantly depend on the amou nt of amyloid deposition around the vessels through the course of illness. (C) 2000 John Wiley & Sons, Inc.