Secondary amyloidosis with severe autonomic dysfunctions

A 46-year-old male underwent hemodialysis because of progressed glomerulo-nephritis. Since he suffered from severe diarrhea during the course of the illness, both gastric and colon biopsies were performed. Significant amyloid deposition was recognized in the submucosal layer of these specimen. This amyloid was positive for anti-AA-protein antibody staining and soluble in KMnO4 solution, indicating secondary induced amyloid. Despite of absence of orthostatic hypotension, examinations revealed extreme reduction in tears and salivary secretion, anhidrosis, a decrease in the coefficiency of variation of the cardiographic R-R interval, and a decrease in the accumulation of [123I]meta-iodobenzylguanidine (MIBG) in the heart, suggesting that severe glandular and visceral autonomic dysfunctions had occurred in the patient.