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14-05-2024 | Post-Infection Chorea | Editor's Choice | News

Corticosteroids, antibiotics, sodium valproate may be of benefit in Sydenham chorea

Author: Dr. Priya Venkatesan

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medwireNews: Treating patients with Sydenham chorea (SC) with immunotherapy, particularly corticosteroids, at first episode results in quick resolution of chorea, while treatment with antibiotics, corticosteroids, or sodium valproate reduces the risk for relapse, suggests a meta-analysis published in JAMA Network Open.

The researchers add, however, that “the mechanism of action for valproate is not fully understood and requires further investigation,” and although most patients achieved a good functional outcome, “specific treatment factors associated with this outcome remain unknown.”

For the meta-analysis, Michael Eyre (King’s College, London, UK) and colleagues collated individual patient data, including treatment, for 1325 patients with SC (68.8% female), who had a median age of SC onset of 10 years. The data were obtained from a systematic search of global scientific literature databases and clinical trial registers from 1945 through 2022.

The researchers investigated three outcomes: chorea duration at the first episode of SC; disease course, defined as either relapsing (at least one relapse) or monophasic (no relapse after at least 24 months’ follow-up); and final functional outcome, defined as either poor (a modified Rankin Scale [mRS] score 2–6 points or persisting chorea or psychiatric or behavioral symptoms at final follow-up) or good (mRS score 0–1 points and no chorea, psychiatric, or behavioral symptoms at any time).

The median duration of chorea at the first episode of SC was 3 months and patients who received immunotherapy were a significant 51% more likely to have a duration shorter than this versus those who did not receive immunotherapy.

In particular, median chorea duration was 1.2 months in 55 patients treated with at least 1 month of steroids, compared with 2.5 months in 55 patients treated with steroids for less than 1 month and 2.8 months in 243 patients not treated with steroids.

Of 766 patients, 34.3% experienced a relapse at least once, with 2.9% relapsing four or more times. Among 189 patients, the median time to relapse was 16.0 months.

The investigators found that an increased risk for relapse was significantly associated with the presence of arthritis or arthralgia (odds ratio [OR]=3.07) and treatment of the first episode with haloperidol (OR=2.02). For patients who were treated with antibiotics, corticosteroids, or sodium valproate, the risk for relapse was reduced by a significant 72%, 68%, and 67%, suggesting an association between these treatments and a monophasic disease course, say Eyre et al.

Of 595 patients with follow-up data, 23.2% had ongoing chorea at final follow-up, while 5.9% of 472 patients had ongoing psychiatric or behavioral problems and 3.0% of 395 patients had ongoing cognitive or school performance problems.

In the researcher’s model for functional outcome, 86.1% of 338 patients had a good functional outcome at the final assessment, but no treatment factors were associated with this outcome. However, factors associated with a poor outcome included receiving treatment with plasma exchange (OR=94.2), having a history of other autoimmune or inflammatory diseases (OR=6.02), and being younger than 5 years of age at disease onset (OR=4.23).

Although they note limitations to their analysis, including the study’s retrospective nature, Eyre et al highlight that their meta-analysis “is the most comprehensive evidence synthesis to date for SC,” and note that it is “forming the base for an ongoing international effort with Delphi methodology to provide consensus-based recommendations for the management of SC.”

medwireNews is an independent medical news service provided by Springer Healthcare Ltd. © 2024 Springer Healthcare Ltd, part of the Springer Nature Group

JAMA Netw Open 2024; 7: e246792

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