Abstract
We report a case of secondary amylodosis in a girl who had been treated for systemic type of juvenile rheumatoid arthritis since she was 3.9 years old. She had intestinal amylodosis and renal amyloidosis when she was 12.2 years old and 13.5 years old. Intestinal amyloidosis was improved with increased corticosteroid, but renal amylodosis exacerbated after the control of JRA. She showed massive proteinuria, microscopic hematuria, and hypertension. Renal biopsy revealed the accumulation of amyloid protein to the glomeruli, tubulointerstitium, and vascular wall. There was mild fibrosis in the interstitium. With the use of the angiotensin converting enzyme inhibitor, massive proteinuria was decreased, and she became normotensive. However, because the increased level of her serum creatinine, together with the finding of the renal biopsy, it was thought that the renal prognosis would be poor.