日本小児循環器学会雑誌 Pediatric Cardiology and Cardiac Surgery

Background: In recent years, acute rheumatic fever (ARF) has been considered an extremely rare condition in Japan, with an incidence of 5–10 cases annually. However, it should be recognized as a serious illness because some patients may develop heart failure (HF) if diagnosis and treatment are delayed.

Methods: The clinical course and prognoses of ARF with carditis in six patients (n＝3 women) diagnosed in our institutions from 1994 to 2018 were investigated by retrospectively reviewing medical records.

Results: The age at diagnosis was 3–13 (median: 8.5) years. The symptoms at onset were fever and arthralgia/arthritis in four, arthralgia/arthritis alone in one, and shortness of breath and fatigability due to HF in one patient. The duration from the initial presentation to the diagnosis of ARF ranged from 3 days to 4 years and 10 months (median: 11.5 days). None of the patients presented with major symptoms other than carditis and polyarthritis based on the revised Jones Criteria. Similar to valvulitis, aortic valve regurgitation (AR), mitral regurgitation (MR), and both AR and MR were observed in 5, 3, and 2 cases, respectively. The patients were treated with prednisolone and/or aspirin and antibiotics (provided as prophylaxis drugs). During follow-up (range: 1–15 [median: 9] years), MR almost disappeared; however, AR remained. Moreover, aortic valve replacement was required in two cases, and recurrence of ARF was not noted.

Conclusion: In this study, arthritis-related symptoms were more likely to be observed during the initial presentation, and the diagnosis of carditis was delayed, resulting in a critical clinical course in some cases. Patients with streptococcal infection-related polyarthritis should be screened for carditis by a pediatric cardiologist.