Preliminary data on prednisone effectiveness in children with Sydenham chorea

Favaretto, Elena; Gortani, Giulia; Simonini, Gabriele; Pastore, Serena; Mascio, Alberto Di; Cimaz, Rolando; Taddio, Andrea

doi:10.1007/s00431-020-03574-y

Cited by 13 publications

(21 citation statements)

References 12 publications

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“…Corticosteroids were prescribed more frequently to patients with carditis, as expected. Furthermore, in agreement with recent findings (19), the majority of patients with chorea received corticosteroids. The burden of the recommended GAS secondary prophylaxis in children is still concerning.…”

Section: Discussionsupporting

confidence: 92%

Acute Rheumatic Fever: Where Do We Stand? An Epidemiological Study in Northern Italy

et al. 2021

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Acute rheumatic fever (ARF) is a non-septic complication of group A β-hemolytic streptococcal (GAS) throat infection. Since 1944, ARF diagnosis relies on the Jones criteria, which were periodically revised. The 2015 revision of Jones criteria underlines the importance of knowing the epidemiological status of its own region with updated data. This study aims to describe ARF features in a retrospective cohort retrieved over a 10-year timespan (2009–2018) and to report the annual incidence of ARF among children in the Province of Monza-Brianza, Lombardy, Italy during the same period. This is a multicentric cross-sectional/retrospective study; 70 patients (39 boys) were diagnosed with ARF. The median age at diagnosis was 8.5 years (range, 4–14.2 years). Overall, carditis represented the most reported major Jones criteria followed by arthritis and chorea (40, 27, and 20 cases, respectively). In order to calculate the annual incidence of ARF, only children resident in the Province of Monza-Brianza were included in this part of the analysis. Therefore, 47 patients aged between 5 and 14 years were identified. The median incidence during the study time was 5.7/100,000 (range, 2.8–8.3/100,000). In the Province of Monza-Brianza, we found an incidence rate of ARF among children aged 5–14 years constantly above the threshold of low-risk area as defined in the 2015 revision of Jones criteria. Therefore, the diagnosis of ARF should be based on the moderate–high-risk set of Jones criteria. However, given the burden of secondary prophylaxis, expert opinion is advisable when the diagnosis of ARF is uncertain.

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Section: Discussionsupporting

confidence: 92%

Acute Rheumatic Fever: Where Do We Stand? An Epidemiological Study in Northern Italy

et al. 2021

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“…In a retrospective observational study from 2020, pimozide acted as a standard therapy against PR, along with valproic acid, showing a response time of 16 days and a remission time of 125 days, both of which were distinctly longer than that of PR (4 and 30 days), furthermore, 3 out of 15 enrolled, to the standard group relapsed, compared to 1 in the PR group (Favaretto et al., 2020).…”

Section: Resultsmentioning

confidence: 99%

Managing and treating Sydenham chorea: A systematic review

et al. 2023

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Introduction Sydenham's chorea (SC), prevalent in developing countries and occasionally affecting developed ones, poses a clinical challenge due to the lack of systematic guidelines for diagnosis and treatment. Resulting from Group A Beta‐Hemolytic Streptococcus infection, SC presents various symptoms. This review aims to collect and evaluate available data on SC management to propose a cohesive treatment plan. Methods We searched PubMed, the Cochrane Library, Google Scholar, and ClinicalTrials.gov for literature on SC management from inception until 24th July 2022. Studies were screened by titles and abstracts. Cochrane Collaboration's Risk of Bias tool (RoB‐1) assessed Randomized Controlled Trials, while the Risk of Bias In Non‐randomized Studies of Interventions (ROBINS‐I) tool evaluated nonrandomized studies. Results The review includes 11 articles assessing 579 patients. Excluding one study with 229 patients, of the remaining 550 patients, 338 (61.5%) were females. Treatments used were dopamine antagonists in 118 patients, antiepileptics in 198, corticosteroids in 134, IVIG in 7, and PE in 8 patients. Dopamine antagonists, particularly haloperidol, were the primary treatment choice, while valproic acid (VPA) was favored among antiepileptics. Prednisolone, a corticosteroid, showed promising results with weight gain as the only side‐effect. Our review emphasizes the importance of immunomodulators in SC, contrasting previous literature. Conclusion Despite limitations, dopamine antagonists can serve as first‐line agents in SC management, followed by antiepileptics. The role of immunomodulators warrants further investigation for conclusive recommendations.

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“…However, there are some side effects of using high doses of corticosteroids including weight gain and acne and in more severe cases progress to Cushing's disease. 4,6 SC pathophysiology involves autoantibodies that bind to basal ganglia neurons and affect their function, IVIG can inactivate these autoantibodies, whereas plasmapheresis will extract them from plasma. Thus, all SC patients who received IVIG or plasmapheresis showed clinical improvement.…”

Section: Discussionmentioning

confidence: 99%

“…1 Various drugs have been used to treat chorea, such as dopamine receptor antagonists (eg, haloperidol), antiepileptic drugs (eg, valproic acid or carbamazepine). [4][5][6][7][8] Studies that examine these drugs in SC patients are very limited and the studies are often observational, objective outcome measures are limited, and the study population is heterogeneous, so the best treatment options are uncertain. We present an Indonesian 10-year-old boy with SC that completely recovered with outpatient management.…”

Section: Introductionmentioning

confidence: 99%

Sydenham Chorea On Indonesian 10 Years Old Boy Caused By Rheumatic Heart Disease : Case Report And Literature Review

Irfana

Airlangga²,

Indrawati³

et al. 2022

MHSJ

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Sydenham's chorea (SC) is one of the manifestations of rheumatic fever, and is the most common cause of chorea in childrens. Sydenham chorea is characterized by involuntary movements such as jerking of the arms, legs, and face. The following case is a 10-year-old boy with complaints of moving his right arm and leg on its own. On physical examination, there was a grade 4/5 systolic murmur at ICS 5 2 cm lateral to the left MCL blowing radiating to the left arm. ASTO examination there is an increase. CT scan of the head without contrast showed no abnormalities. The results of echocardiography showed severe mitral regurgitation. The patient was diagnosed with Sydenham Chorea and Rheumatic Heart Disease. The patient refused hospitalization and was treated as an outpatient with given therapy erythromycin 250 mg four times daily for 10 days followed by a twice daily dose for the next two months, symptomatic haloperidol 1 mg and trihexyphenidyl 0.5 mg twice a day, aspirin 300 mg four times a day for one month. From this treatment, the complaints improved slowly, and the chorea disappeared within 10 days of the start of treatment.

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Preliminary data on prednisone effectiveness in children with Sydenham chorea

Cited by 13 publications

References 12 publications

Acute Rheumatic Fever: Where Do We Stand? An Epidemiological Study in Northern Italy

Acute Rheumatic Fever: Where Do We Stand? An Epidemiological Study in Northern Italy

Managing and treating Sydenham chorea: A systematic review

Sydenham Chorea On Indonesian 10 Years Old Boy Caused By Rheumatic Heart Disease : Case Report And Literature Review

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