Pharmacotherapy for Sydenham’s chorea: where are we and where do we need to be?

ABSTRACT

Introduction

Sydenham’s chorea (SC) is the most common cause of acquired chorea in children. The existing literature describes it as a benign, self-remitting condition. However, recent evidence discloses the persistence of long-course neuropsychiatric and cognitive complications in adulthood, which imposes to redefine the concept of ‘benignity’ of such condition. In addition, therapies are mostly empirical and non-evidence based.

Areas covered

Here, we conducted an electronic exploration of the PubMed database and selected 165 relevant studies directly correlated to SC treatment. Critical data from selected articles were synthesized to provide an update on pharmacotherapy in SC, which basically consists of three pillars: antibiotic, symptomatic and immunomodulant treatments. Moreover, since SC mostly affects females with recurrences occurring in pregnancy (chorea gravidarum), we focused on the management in pregnancy.

Expert opinion

SC is still a major burden in developing countries. The first therapeutic strategy should be the primary prevention of group A beta-hemolytic streptococcal (GABHS) infection. Secondary antibiotic prophylaxis should be performed in every SC patient as the World Health Organization (WHO) guidelines recommend. Symptomatic or immunomodulant treatments are administered according to clinical judgment. However, a greater effort to understand SC physiopathology is needed, together with larger trials, to outline appropriate therapeutic indications.

KEYWORDS:

• Sydenham’s chorea
• rheumatic fever
• autoimmune movement disorders
• treatment
• hyperkinetic movement disorders
• chorea gravidarum

Article highlights

• Sydenham’s chorea (SC) is the most common acquired chorea in children worldwide; still, its therapeutic strategies are empirical and non-evidence based.

• Current pharmacotherapy for SC basically consists of three pillars: antibiotic, symptomatic and immunomodulant medications.

• Antibiotic prophylaxis should be initiated as soon as SC is diagnosed, according to the WHO guidelines, to prevent beta-hemolytic streptococcal (GABHS) re-infection and reduce the risk of cardiac involvement.

• Symptomatic treatments, which consist in anti-seizure medications (ASMs) and dopamine depleting agents, should be considered when symptoms are clinically relevant; however, no consensus exist on which should be the first choice.

• Immunomodulant strategies are gaining increasingly attention given the autoimmune pathogenesis of SC, with promising results.

• There are numerous gaps and unmet needs in SC management and treatment, which highlights the necessity of a deeper comprehension of its pathogenesis and sizable controlled studies to define standardized guidelines.

Declaration of interest

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

Reviewer disclosures

Peer reviewers on this manuscript have no relevant financial or other relationships to disclose.

Additional information

Funding

This paper was not funded.