ISSN:
1432-198X
Keywords:
Juvenile chronic arthritis
;
Renal amyloidosis
;
Chlorambucil
;
Electron microscopy
;
Protein AA
Source:
Springer Online Journal Archives 1860-2000
Topics:
Medicine
Notes:
Abstract Over a 22-year period, eith patients affected with severe systemic or polyarticular juvenile chronic arthritis (JCA) developed systemic amyloidosis with nephrotic syndrome. They were treated with chlorambucil over 5–192 months (mean=44 months). With treatment, an abrupt decrease in the severity of JCA was observed in six patients but two patients were chlorambucil resistant. After a mean follow-up period of 10 years from onset of renal symptoms, one chlorambucil-resistant patient died of end-stage renal failure; two patients have a persistent nephrotic syndrome; and five patients are free from proteinaria, of whom one has developed hypertension. A good correlation was observed between the response of the rheumatic disease to chlorambucil treatment and the clinical course of renal symptoms. Fourteen renal biopsies were performed in these eight patients. In all, amyloid deposits were of the AA type, which persisted on repeat biopsies. In addition, 15%–60% of glomeruli had become globally sclerotic by the second or third biopsies. At the ultrastructural level, modifications in the structure of amyloid deposits and reparative changes of the glomeruli, characterized by partial restoration of glomerular architecture, were observed in three patients with a favourable clinical course.
Type of Medium:
Electronic Resource
URL:
http://dx.doi.org/10.1007/BF00869821
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