Acute rheumatic fever — a late complication of streptococcal infection: results of a 25-year study

Despite a decline in incidence, acute rheumatic fever (ARF) and recurrent ARF is a late autoimmune complication of S. pyogenes infection — remain a significant public health problem, being a cause of chronic rheumatic heart disease.

Objective: to provide a clinical and epidemiological characterization and describe the outcomes of ARF based on observations of patients hospitalized between 2001 and 2025 at the Morozovskaya children’s city clinical hospital of the Moscow Department of Health.

Materials and methods. We observed 113 children (60 girls, 53 boys), aged 4—17 years (median age 10 [8; 13] years), with a diagnosis of ARF (101 children) or recurrent ARF (12 children). We assessed clinical and anamnestic data, laboratory test results (antistreptolysin-O (ASO), C-reactive protein), electrocardiography (ECG), and echocardiography. Children with Sydenham's chorea underwent additional examinations.

Results. A high rate of underdiagnosis of ARF was identified (63.7%). The onset of ARF most commonly occurred during the winter-spring period (61.9 %). To confirm markers of previous streptococcal infection, measuring ASO levels is more informative; an elevated ASO level (median 795 [385; 1440] IU/mL) was detected in 86.7% of children. In theclinical presentation of ARF, carditis was the predominant manifestation (81.4 %), and its frequency increased with age (p = 0,003). The mitral valve (MV) was most commonly affected (54%); combined involvement of the MV and aortic valve (AV) was observed in 34.5% of children, while isolated AV involvement occurred in 11.5 %. Arthritis was detected in 44.2% of children, with a high frequency (36/50, 72%) of atypical joint syndrome. Our study recorded a high incidence of Sydenham's chorea (56 children, 49.6%), including isolated Sydenham's chorea in 30.4%. The incidence of Sydenham's chorea decreased with age (p = 0,004). Rare manifestations of ARF — erythema marginatum and subcutaneous nodules — were observed in 14.2% and 3.4% of patients, respectively. A high incidence of chronic rheumatic heart disease (42%) was noted, with the development of rheumatic heart valve defects in 38% of cases.

Conclusion. The study highlights the need for vigilance regarding ARF in patients following streptococcal infection to prevent severe outcomes such as the development of acquired heart valve defects.

1. Gewitz MH, Baltimore RS, Tani LY, Sable CA, Shulman ST, et al. American Heart Association Committee on Rheumatic Fever, Endocarditis, and Kawasaki Disease of the Council on Cardiovascular Disease in the Young. 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—1818. doi: 10.1161/CIR.0000000000000205.

2. Ralph AP, Noonan S, Wade V, Currie BJ. The 2020 Australian guideline for prevention, diagnosis and management of acute rheumatic fever and rheumatic heart disease. Med J. 2021;214:220—227. doi: 10.5694/mja2.50851.

3. Belov BS. Acute rheumatic fever: current state of the problem. Russian Medical Journal. 2004;6:418. (In Russ).

4. Stephenson Z, Jones B, Remenyi B, de Dassel J, Dunn S, Francis JR, Yan J. Acute Rheumatic Fever and Rheumatic Heart Disease in Children Aged Less Than 5 Years in the Northern Territory Between 2010 and 2020. J Paediatr Child Health. 2025 Dec;61(12):1850—1856. doi: 10.1111/jpc.70194.

5. Ali Sulafa KM, Karrar ZA, Elkurdufani N, Ibrahim N. Sudan's rheumatic fever and rheumatic heart disease guidelines: a simplified approach in an endemic country. Front Cardiovasc Med. 2024 May 10;11:1403131. doi: 10.3389/fcvm.2024.1403131.

6. McDonald M, Currie BJ, Carapetis JR. Acute rheumatic fever: a chink in the chain that links the heart to the throat? Lancet Infect Dis. 2004 Apr;4(4):240—245. doi: 10.1016/S1473-3099(04)00975-2.

7. Deev IA, Kobyakova OS, Starodubov VI, Aleksandrova GA, Golubev NA, et al. Incidence of diseases among the child population of Russia (0—14 years old) in 2023 with a diagnosis established for the first time in life: statistical materials. Moscow: FSBI «CRIOIH» of the Ministry of Health of the Russian Federation; 2023. 156 p. DOI: 10.21045/978-5-94116-163-8-2024. (In Russ).

8. Deev IA, Kobyakova OS, Starodubov VI, Aleksandrova GA, Golubev NA, et al. Overall morbidity among the child population of Russia (0—14 years old) in 2023: statistical materials. Moscow: FSBI «CRIOIH» of the Ministry of Health of the Russian Federation; 2023. 156 p. DOI: 10.21045/978-5-94116-164-5-2024. (In Russ).

9. Deev IA, Kobyakova OS, Starodubov VI, Aleksandrova GA, Golubev NA, et al. Incidence of diseases among the adolescent population of Russia (15—17 years old) in 2023 with a diagnosis established for the first time in life: statistical materials. Moscow: FSBI «CRIOIH» of the Ministry of Health of the Russian Federation; 2023. 158 p. DOI: 10.21045/978-5-94116-167-6-2024. (In Russ).

10. Deev IA, Kobyakova OS, Starodubov VI, Aleksandrova GA, Golubev NA, et al. Overall morbidity among the adolescent population of Russia (15—17 years old) in 2023: statistical materials. Moscow: FSBI «CRIOIH» of the Ministry of Health of the Russian Federation; 2023. 158 p. DOI: 10.21045/978-5-94116-168-3-2024. (In Russ).

11. de Loizaga SR, Arthur L, Arya B, Beckman B, Belay W, et al. Rheumatic Heart Disease in the United States: Forgotten But Not Gone: Results of a 10 Year Multicenter Review. J Am Heart Assoc. 2021 Aug 17;10(16): e020992. doi: 10.1161/JAHA.120.020992.

12. Salikhova MZ, Nazarova ID, Avezova ZSh. Clinical and immunological characteristics of acute rheumatic fever in children. Healthcare of Tajikistan. 2024;(S3). Available from: https://cyberleninka.ru/article/n/kliniko-immunologicheskaya-harakteristika-ostroy-revmaticheskoy-lihoradki-u-detey (cited 2026 Mar 29). (In Russ).

13. Goyal A, Vaideeswar P, Daga P, Bhargav R. Acute rheumatic fever — A pathological analysis of clinically missed cases. Indian J Pathol Microbiol. 2021 Oct-Dec;64(4):651—654. doi: 10.4103/IJPM.IJPM_1422_20.

14. Parks T, Kado J, Colquhoun S, Carapetis J, Steer A. Underdiagnosis of acute rheumatic fever in primary care settings in a developing country. Trop Med Int Health. 2009 Nov;14(11):1407—1413. doi: 10.1111/j.1365-3156.2009.02385.x.

15. Laloğlu F, Ceviz N. Changes in the frequency and clinical features of acute rheumatic fever in the COVID-19 era: a retrospective analysis from a single center. Rev Assoc Med Bras (1992). 2022 Sep;68(9):1313—1317. doi: 10.1590/1806-9282.20220620.

16. Erdem S, Demir F, Ayana M, Canan O, Okuducu YK, Arslan A, Kucukosmanoglu O, Özbarlas N. Acute rheumatic fever in south-east of Turkey: clinical features and epidemiological evaluation of the patients over the last 25 years. Cardiol Young. 2020 Aug;30(8):1086—1094. doi: 10.1017/S1047951120001596.

17. Ghamrawy A, Ibrahim NN, Abd El-Wahab EW. How accurate is the diagnosis of rheumatic fever in Egypt? Data from the national rheumatic heart disease prevention and control program (2006—2018). PLoS Negl Trop Dis. 2020 Aug 17;14(8):e0008558. doi: 10.1371/journal.pntd.0008558.

18. Alberio AMQ, Pieroni F, Di Gangi A, Cappelli S, Bini G, et al. Toward the Knowledge of the Epidemiological Impact of Acute Rheumatic Fever in Italy. Front Pediatr. 2021 Dec 15;9:746505. doi: 10.3389/fped.2021.746505.

19. Kumar M, Little J, Pearce S, MacDonald B, Greenland M, et al. Clinical profile of paediatric acute rheumatic fever and rheumatic heart disease in Western Australia: 1987 to 2020. J Paediatr Child Health. 2024 Aug;60(8): 375—383. doi: 10.1111/jpc.16617.

20. Sadiq NM, Afshan G, Qureshi AU, Sadiq M. Current Clinical Profile of Acute Rheumatic Fever and Recurrent Acute Rheumatic Fever in Pakistan. Pediatr Cardiol. 2024 Feb;45(2):240—247. doi: 10.1007/s00246-023-03378-5.

21. Robazzi TC, de Araújo SR, Costa Sde A, de Oliveira Júnior AB, Nunes LS, Guimarães I. Articular manifestations in patients with atypical rheumatic fever. Rev Bras Reumatol. 2014 Jul-Aug;54(4):268—272. doi: 10.1016/j.rbr.2014.02.006.

22. EL Banna HH, Swelam RA. Clinical presentations of atypical arthritis in Egyptian children with acute rheumatic fever. J Am Sci. 2013;9:253—261.

23. Kantemirova MG, Novikova YuYu, Korovina OA, Drozdova IM, Glazirina AA, Ovsyannikov DYu. Modern features of rheumatic chorea in children. Pediatria n.a. G.N. Speransky. 2016;95(3):15—22. (In Russ).

24. Ovsyannikov DYu, Kantemirova MG, Illarionova TYu, Paunova SS. Ahead of his time… (on the 160th anniversary of А.А. Kisel birth). Pediatria n.a. G.N. Speransky. 2019;98(5):257—260. (In Russ).