Thu0510 chorea and Carditis: An Unexpected Combination. The New Face of Acute Rheumatic Fever

Abstract

Background:Acute Rheumatic Fever (ARF) is an immunomediated multisystem disease that occurs about 2-5 weeks after Group A Streptococcus Pyogenes beta-hemolytic (GAS) pharyngitis. After a negative peak in the 1980s, following the introduction of antibiotic prophylaxis, the disease is currently recovering. Rheumatic carditis is one of the most worrying aspects as it is still one of the major causes of cardiovascular death in the young-adult population. However, if diagnosed early and treated, sequelae with aortic and mitral valve involvement can be prevented.Objectives:The aim of our study is a description of Acute Rheumatic Fever in all its manifestations in a cohort of pediatric patients belonging to the Azienda Ospedaliera Universitaria Integrata of Verona.Methods:A retrospective analysis was conducted collecting all the cases of ARF, diagnosed by Jones’s criteria, related to Pediatric Rheumatology and Pediatric Cardiology of Verona from January 2005 to December 2019. Demographic and clinical data were collected for all patients such as clinical presentation, disease evolution and cardiac involvement.Results:73 patients were analyzed, of whom 53 had an acute onset of ARF and 20 received a diagnosis of previous ARF due to indolent carditis. The prevalent age at the time of diagnosis in both groups was between 5 and 14 years of age. Among patients with acute onset, carditis was the most frequent major manifestation (94.3%), followed by polyarthritis (41.5%), chorea (24.5%) and erythema marginatum (7.5%). Only in one patient we could observe subcutaneous nodules (1.8%). Regarding the minor manifestations, the increase in inflammation markers was present in 83% of cases and fever was present in 75.5%, followed by arthralgia (58.4%) and prolonged of PR interval to ECG (9.4%). Carditis was also present in all 13 patients who presented chorea. Clinically, previously unknown heart murmur occurred in 28 patients. Therefore, the mismatch between cardiac objectivity and carditis finding is clear: infact, compared to an important finding of carditis (50 patients) only slightly more than half of the patients (28 patients) showed an evident clinic finding. Finally, no correlation was found between the levels of the antistreptolysin O titer and the severity of heart damage. Patients with early diagnosis of carditis were treated at onset with corticosteroid therapy according to the American Heart Association scheme and did not show valvular cardiac outcomes. A patient who received a late diagnosis of carditis currently presents a significant and permanent cardiac damage despite adequate steroid treatment undertaken at the time of diagnosis.Conclusion:The description of this cohort of pediatric patients shows that the ARF has not disappeared in industrialized countries. Treatment of streptococcal infection (primary prophylaxis) plays a key role in preventing ARF. Of great impact is the prevalence of carditis which is present in 94.3% of patients. Early diagnosis is therefore of primary importance and the subsequent follow-up path, consisting of periodic therapy with penicillin (secondary prophylaxis) and periodic cardiological checks, greatly affects children’s quality of life. Chorea, unlike what has been described in the literature, occurred simultaneously with the cardiac process, while the cutaneous manifestations (subcutaneous nodules and erythema marginatum), once pathognomonic of the rheumatic disease, are today of rare observation.References:[1]Carapetis, Beaton, Cunningham et al. Acute rheumatic fever and rheumatic heart disease. Nature Reviews Disease Primers. 2016;2:15084.Disclosure of Interests:None declared