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

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.

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.

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.