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Prednisone shortens intensity and duration in Sydenham’s chorea compared with placebo.

Question

  • In a patient with Sydenham’s chorea due to rheumatic fever, does the use of prednisone, compared with placebo, shorten the intensity and duration of the chorea.

Clinical Bottom Lines

1. This RCT demonstrates that in patients with Sydenham’s chorea prednisone does shorten the intensity and duration of their chorea symptoms compared to placebo.
2. There was a greater reduction in the chorea intensity scale in the prednisone group, notable as early as week 1.
3. Percentage decrease in the chorea intensity scale score was persistently and significantly greater in the prednisone group.
4. Chorea complete remission time with prednisone was significantly shorter when compared with the placebo group (54.3 days vs. 119.9 days).
5. It does not, however, show that prednisone decreases the rate of recurrence of Sydenham’s chorea.


Summary of Key Evidence

  1. Thirty-seven children (ages 7-15) who met the Jones criteria for rheumatic fever and had Sydenham’s chorea were randomized to receive either prednisone or placebo.
  2. The mean chorea intensity score was measured at weeks 1-4 and then weeks 8 and 12.
  3. Inclusion criteria: within 90 days of initiation of chorea and a diagnosis of rheumatic fever based on Jones criteria.
  4. Exclusion criteria: another possible cause of chorea could be identified, there was a contraindication to immunosuppression, or the patient had a co-morbidity that would have been exacerbated with prednisone therapy (acute psychiatric illness, GI illness, hyperglycemia, septicemia, or hypertension). Additionally if the patient had evidence of acute Carditis, which is an inconsistency given that patients with carditis were included in both groups.
  5. No statistical difference in the rate of relapse between the two groups when followed for 1-4 years after remission. Summarize the key evidence so that others can see it for themselves.

Additional Comments

  • Antibodies produced against M proteins in a strep infection appear to cross react with the basal ganglia causing Sydenham’s chorea in patients with rheumatic fever. This suggests that an immune-modulating therapy, such as steroids, would be beneficial. 2
  • Two patients were excluded from the study after randomization and these were not analyzed in an intention to treat analysis.1
  • While the rate of relapse was stated there was no information provided in regards to how long between initial event and relapse.1

Citation

1. Paz, JA, Silva, CA, Marques-Dias, MJ. Randomized double-blind study with prednisone in Sydenham's chorea. Pediatric Neurology 2006; 34:264.
2. Church, AJ, Cardoso, F, Dale, RC, et al. Anti-basal ganglia antibodies in acute and persistent Sydenham's chorea. Neurology 2002; 59:227.

CAT Author: Jessica Foley, MD

CAT Appraisers: Francis McBee Orzulak, MD

Date appraised: March 25, 2009

Last updated June 11, 2009
Department of Pediatrics and Communicable Diseases
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