2002 Volume 112 Issue 4 Pages 385-391
The patient was a 48-year-old female. She had had Raynaud’s phenomenon since 1986. In 1988, she developed edematous sclerosis of the hands and trismus, and she diagnosed with systemic sclerosis (SSc). Then small ulcers repeatedly developed on both feet and were associated with reflux esophagitis. In 1998, biopsy revealed deposition of amyloid, a protein in the duodenum, leading to a diagnosis of secondary amyloidosis. In 1999, amyloid deposition was also detected in the rectum. She developed cardiac failure and renal failure in December, leading to hemodialysis. Her cardiac function recovered to normal, but her intestinal movement was markedly reduced. In February of 2000, she suddenly exhibited cardiopulmonary standstill and died. Autopsy revealed severe deposition of amyloid in the heart, many small fibroid foci, and necrotic foci. There was also severe deposition of amyloid in the kidneys and concentric intimal hypertrophy of small arteries in the kidneys. Moreover, fibrosis of the internal circular muscle layer was observed from the esophagus to the colon, with severe deposition of amyloid. Although SSc is rarely complicated by secondary amyloidosis, we think it necessary to take into consideration the complications of secondary amyloidosis in the treatment of patients with long-term SSc.
