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- Title
Treatments and Outcomes Among Patients with Sydenham Chorea: A Meta-Analysis.
- 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
- Abstract
Key Points: Question: Which clinical and treatment factors at onset of Sydenham chorea are associated with chorea duration, relapsing disease course, and functional outcome? Findings: In this individual patient data meta-analysis of 1479 patients, those receiving at least 1 month of corticosteroids had a median chorea duration of 1.2 months vs 2.8 months for patients receiving none, a significant difference. Patients treated with antibiotics, corticosteroids, or sodium valproate had significantly reduced odds of relapse. Meaning: These observational data support the use of corticosteroids, antibiotics, and sodium valproate for treatment of Sydenham chorea. 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: [Sydenham OR Sydenham's OR rheumatic OR minor] AND chorea). 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 <.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. This individual patient data meta-analysis investigates historical changes in the clinical characteristics of Sydenham chorea and identifies clinical and treatment factors at disease onset associated with chorea duration, relapsing disease course, and functional outcome among patients with the disorder.
- Subjects
CHOREA; ADRENOCORTICAL hormones; ANTIBIOTICS; DISEASE duration; DATA analysis; STATISTICAL hypothesis testing; RESEARCH funding; FUNCTIONAL assessment; IMMUNOTHERAPY; FISHER exact test; LOGISTIC regression analysis; HOSPITAL care; META-analysis; TREATMENT duration; CHI-squared test; MANN Whitney U Test; MULTIVARIATE analysis; AGE factors in disease; ODDS ratio; KAPLAN-Meier estimator; LOG-rank test; VALPROIC acid; STATISTICS; DISEASE relapse; HEALTH outcome assessment; CONFIDENCE intervals; DATA analysis software; CLINICAL trial registries; PATIENT aftercare; SENSITIVITY &; specificity (Statistics); PLASMA exchange (Therapeutics); DISEASE risk factors; SYMPTOMS
- Publication
JAMA Network Open, 2024, Vol 7, Issue 4, pe246792
- ISSN
2574-3805
- Publication type
Article
- DOI
10.1001/jamanetworkopen.2024.6792