Treatments and Outcomes Among Patients with Sydenham Chorea
Authors: Eyre Michael , Thomas Terrence , Ferrarin Emanuela , Khamis Sonia , Zuberi Sameer M. , Sie Adrian , Newlove-Delgado Tamsin , Morton Michael , Molteni Erika , Dale Russell C. , Lim Ming , Nosadini Margherita ,   Sydenham’s Chorea Systematic Literature Review Working Group, Aty-Marzouk Pakinam A , Cardoso Francisco , Cavalcanti André , Galloway Yvonne , Jack Susan , Maia Debora , Marino Achille , Orsini Alessandro , Sartori Stefano 
Published: April 2024

Importance

Sydenham chorea is the most common acquired chorea of childhood worldwide; however, treatment is limited by a lack of high-quality evidence.

Objectives

To evaluate historical changes in the clinical characteristics of Sydenham chorea and identify clinical and treatment factors at disease onset associated with chorea duration, relapsing disease course, and functional outcome.

Data Sources

The systematic search for this meta-analysis was conducted in PubMed, Embase, CINAHL, Cochrane Library, and LILACS databases and registers of clinical trials from inception to November 1, 2022 (search terms: [SydenhamORSydenham’sORrheumaticORminor] ANDchorea).

Study Selection

Published articles that included patients with a final diagnosis of Sydenham chorea (in selected languages).

Data Extraction and Synthesis

This study followed the Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) reporting guideline. Individual patient data on clinical characteristics, treatments, chorea duration, relapse, and final outcome were extracted. Data from patients in the modern era (1945 through 2022) were entered into multivariable models and stratified by corticosteroid duration for survival analysis of chorea duration.

Main Outcomes and Measures

The planned study outcomes were chorea duration at onset, monophasic course (absence of relapse after ≥24 months), and functional outcome (poor: modified Rankin Scale score 2-6 or persisting chorea, psychiatric, or behavioral symptoms at final follow-up after ≥6 months; good: modified Rankin Scale score 0-1 and no chorea, psychiatric, or behavioral symptoms at final follow-up).

Results

In total, 1479 patients were included (from 307 articles), 1325 since 1945 (median [IQR] age at onset, 10 [8-13] years; 875 of 1272 female [68.8%]). Immunotherapy was associated with shorter chorea duration (hazard ratio for chorea resolution, 1.51 [95% CI, 1.05-2.19];P = .03). The median chorea duration in patients receiving 1 or more months of corticosteroids was 1.2 months (95% CI, 1.2-2.0) vs 2.8 months (95% CI, 2.0-3.0) for patients receiving none (P = .004). Treatment factors associated with monophasic disease course were antibiotics (odds ratio [OR] for relapse, 0.28 [95% CI, 0.09-0.85];P = .02), corticosteroids (OR, 0.32 [95% CI, 0.15-0.67];P = .003), and sodium valproate (OR, 0.33 [95% CI, 0.15-0.71];P = .004). Patients receiving at least 1 month of corticosteroids had significantly lower odds of relapsing course (OR, 0.10 [95% CI, 0.04-0.25];P &amp;lt; .001). No treatment factor was associated with good functional outcome.

Conclusions and Relevance

In this meta-analysis of treatments and outcomes in patients with Sydenham chorea, immunotherapy, in particular corticosteroid treatment, was associated with faster resolution of chorea. Antibiotics, corticosteroids and sodium valproate were associated with a monophasic disease course. This synthesis of retrospective data should support the development of evidence-based treatment guidelines for patients with Sydenham chorea.