T. Fujito et al., SYSTEMIC AMYLOIDOSIS FOLLOWING ANKYLOSING-SPONDYLITIS ASSOCIATED WITHCONGESTIVE-HEART-FAILURE - A CASE-REPORT, Japanese Heart Journal, 36(5), 1995, pp. 681-688
We report the case of a 38-year-old man who developed fatal, systemic
amyloidosis following ankylosing spondylitis. He was admitted for symp
toms of congestive heart failure. Based on parotid gland biopsy and ec
hocardiography, he was diagnosed as having systemic amyloidosis follow
ing active ankylosing spondylitis. However, the clinical course was ra
pidly progressive and eventually the patient died of acute necrotizing
pancreatitis. The association has been reported thus far in a limited
number of cases worldwide. The literature has featured localized lesi
ons and a benign clinical course of the amyloidosis. This case, the fi
rst report from Japan, indicates that the amyloidosis associated with
ankylosing spondylitis might exhibit a rapidly progressive clinical co
urse, thereby suggesting that in such a case, meticulous treatment is
required.