2016 Volume 89 Issue 1 Pages 58-61
Amyloidosis is a relatively rare disorder, characterized by amyloid deposition in various organs, disrupting tissue structure and function. We report a case of AA amyloidosis with polymyalgia rheumatica (PMR) . The patient was an 80-year-old woman with a history of chronic lower abdominal pain, nausea, and constipation. She was referred to our hospital for raised inflammatory markers (CRP 20 mg/dL and WBC 24520/µL) . Abdominal CT revealed iliopsoas abscess resulting from sigmoid colon perforation. She was treated with CT-guided drainage, antibiotics, and total parenteral nutrition (TPN) instead of emergency surgery because of her poor general condition on admission. She suffered from catheter infection, candidemia, candida endophthalmitis, cholecystitis, and pseudomembranous colitis during the treatment and experienced nausea, vomiting, and abdominal pain after starting oral nutrition. The diagnosis of AA amyloidosis was confirmed by endoscopic biopsies. She underwent colostomy on day 271. She still suffered from severe infection after the surgery, and immunosuppressive treatment was not indicated. She had progressive malabsorption due to gastrointestinal amyloidosis and died on day 425.
The prognosis of gastrointestinal perforation with amyloidosis is poor because of the severity of underlying illnesses, infections, and postoperative leakage. Serum AA levels must be reduced to prevent gastrointestinal amyloidosis caused by AA amyloidosis. Tocilizumab, a humanized anti-interleukin-6 receptor antibody, is a potential treatment option for AA amyloidosis.
