Acute rheumatic fever: new aspects of an old disease

Background: Acute rheumatic fever is a nonsuppurative sequela that occurs two to four weeks following group A beta-hemolytic Streptococcus (GABHS) pharyngitis. Despite its decline in incidence in Europe, rheumatic fever still represents a serious healthcare concern. The most common manifestations are arthritis and carditis, followed by chorea, erythema marginatum, and subcutaneous nodules. The diagnosis of ARF is clinical and requires satisfaction of revised Jones criteria as well as evidence of a recent streptococcal infection. Objectives: Aim of this study was to highlight new emerging features of disease clinical presentation. Methods: Data regarding patients who fulfilled the Revised Jones Criteria for acute rheumatic fever were retrospectively collected from and July 2017. The following clinical and demographic data were obtained: patient sex, birth date, age at presentation, clinical manifestations, evolution of cardiac involvement after therapy. Results: 35 patients (19 males, 16 females) were included. The mean age at diagnosis 7,9±4 years (range 2–17). Carditis was the most frequent manifestation, occurring in 31 patients (89%). Other common symptoms were fever (91%), artrhtiris (43%) or arthralgias (54%) and chorea (20%). In 6 out of 7 patients chorea occurred together with carditis. Cardiac auscultation revealed new pathologic murmurs only in 49% of cases. Echocardiography detected cardiac involvement in the absence of auscultatory findings (subclinical carditis) in 11 patients at the time of diagnosis. In 3 cases ultrasound examination became positive within 4 weeks. Throat cultures were negative in 37% of cases. Anti-streptolysin O (ASO) and anti-desoxyribonuclease B (ADB) antibodies titers did not bear correlation with the severity of cardiac involvement. Conclusions: In our casistic, carditis was the most common and worrysome manifestation of ARF. Detection of cardiac involvement is still challenging and echocardiography testing should be performed even in the absence of abnormal auscultation findings. If the initial ultrasound is negative but clinical suspiciun remains high, US investigation should be repeated, given that rheumatic carditis can evolve over weeks to months. Early detection and treatment of manifestations improves outcome as in all the cases of this series. Streptococcal serology is most helpful in the diagnosis of ARF because culture or detection of the organism is usually no longer possible by the time ARF presents. The severity of clinical manifestations, particularly of carditis, does not correlate strictly with ASO and ADB titer. Sydenham chorea, which usually has a longer latent period (up to 6 months), tends to occur earlier in the disease course, presenting in the acute phase in this series. References: Gewitz MH, et al. Revision of the Jones Criteria for the Diagnosis of Acute Rheumatic Fever in the Era of Doppler Echocardiography A Scientific Statement From the American Heart Association. Circulation. 2015 May 19;131(20):1806–18. Parks T, et al. ASO titer or not? When to use streptococcal serology: a guide for clinicians. Eur J Clin Microbiol Infect Dis. 2015 May;34(5):845–9. Disclosure of Interest: None declared DOI: 10.1136/annrheumdis-2018-eular.4725 Citation: Ann Rheum Dis, volume 77, supplement Suppl, year 2018, page A1656Session: Paediatric rheumatology