ABSTRACT

Patients with Familial Amyloidotic Polyneuropathy (FAP) show various clinical symptoms reflecting peripheral circulatory disturbances. Orthostatic hypotension is one of the most common and serious complications in these patients. The integral role of the arterioles in the regulation of blood pressure to orthostatic stress is well established. Although change in the function of venules may also be found, it has not been well examined in those patients. Attention has been focused on near-infrared spectrophotoscopy as a non-invasive tool to assess the venules. This chapter aims to evaluate morphological changes in the skin venules of the fingers in patients with FAP using near-infrared spectrophotoscopy, and discusses the utility of this method. Three major factors, amyloid deposition around the vessels, the dysfunction of smooth muscles, and dynamic changes in blood velocity accompanied by orthostatic hypotension may contribute to the phenomenon of the venules. However, the amount of perivascular amyloid deposition in the venules is usually smaller than that of the arterioles in FAP.