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Article Summary
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Immonen K et al. - The outcome of Juvenile Idiopathic Arthritis (JIA)-associated amyloidosis is poor. However, renal disease regressed in some patients under vigorous treatment. Methods- Aim was to determine the outcome of amyloidosis associated with JIA
- Patient registers and amyloidosis biopsy files were scrutinized from 1976-2003 to look for amyloidosis in pts under age 19y
- Medical records were reviewed and pts were interviewed by telephone
- The causes of any deaths were obtained from death certificates
Results- 16/24 pts (67%) had proteinuria, but none had renal insufficiency
- The 5-y survival rate of the series was 87.5%, and 10-y survival was 75%
- 10/24 pts (42%) died during a mean f/u of 15.4y
- The main cause of death was related to JIA in all pts but one
- Pts treated with prednisolone alone from the diagnosis of amyloidosis onward had a mortality rate higher than those taking DMARDs and/or cytostatics
- At the end of f/u, 14 pts (58%) were alive, 12 with normal renal function 1 had renal insufficiency, and 1 proteinuria
- Proteinuria disappeared in 4 pts who were proteinuric at baseline, and their renal function remained normal
- All the live pts had completed at least the 9 yrs of compulsory education, and 4 had academic degrees
- Two female pts had delivered healthy children
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