Moderate to severe Sydenham chorea: management when first-line corticosteroid immunotherapy has not achieved functional recovery
Clinical scenario
This protocol applies to patients with moderate to severe Sydenham chorea (modified Rankin Scale score 2–5). These patients present with motor symptoms — with or without behavioral or psychiatric features — that are disrupting activities of daily living, school, and family life. At this severity, immunotherapy is indicated.
Previous treatment — failure condition
First-line management comprises antibiotic therapy at presentation, first-line corticosteroid immunotherapy, and symptomatic pharmacotherapy for the movement disorder.
This protocol is for patients in whom that course has not achieved the expected goals: functional recovery with resolution of chorea and behavioral or psychiatric symptoms, as assessed within 2–4 weeks of treatment initiation (or earlier in severe cases).
Next-line approach (partial overview)
When first-line corticosteroids have not produced adequate response, additional immunotherapy may be considered — options include intravenous immunoglobulin or therapeutic plasma exchange.
The complete regimen, sequencing criteria, and selected-patient considerations are in the full structured protocol below.
Treatment goals
Resolution of chorea and return to normal physical, educational, and social functioning.
References
- DOI: 10.1542/peds.2025-072466
- Patients with moderate and severe SC should be offered immunotherapy.
- Motor with/without behavioral or psychiatric symptoms with impact to activities of daily living, school and family life but not fulfilling criteria as in Severe Disease below.
- In moderate to severe SC and a failure of response to corticosteroid therapy, IVIG or plasma exchange can be considered as additional immunotherapy.
- Depending on severity and treatment response, prolonged use of corticosteroids may be considered, such as monthly IV methylprednisolone for 1–3 mos or oral prednisone/prednisolone for 1–3 mos.
- The main goal of management is resolution of SC symptoms and an early return to normal physical, educational, and social functioning, where possible.
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