Tocilizumab for juvenile Takayasu arteritis complicated with acute heart failure at onset

Chronic heart failure caused by aortic valve regurgitation is a common complication of Takayasu arteritis (TA). However, fewer patients develop acute heart failure (AHF), and no specific treatment for AHF in TA has been established. We encountered a 12-year-old girl with TA who developed AHF at the onset. We successfully treated her with intravenous methylprednisolone and tocilizumab. She developed palpitations and shortness of breath 3 weeks before admission. Her symptoms exacerbated rapidly and she finally entered the intensive care unit due to respiratory distress and tachycardia. Blood pressure measurements on the left arm and bilateral legs were paradoxically lower than that on the right arm. Chest X-ray revealed a severely enlarged heart. Contrast computed tomography showed an expanded aorta, aortic aneurysm, meandering, and irregular diameter of the aorta. The left ventricular ejection fraction was 20% on cardiac ultrasound. Her medical condition was finally diagnosed as TA with AHF. Along with inotropes and diuretics, methylprednisolone pulse therapy was administered on hospital days 2-4 and hospital days 12-14, followed by oral prednisolone. However, cardiac function was not notably improved. As intravenous cyclophosphamide therapy requires hydration and may exacerbate AHF, we initiated weekly subcutaneous tocilizumab treatment (162 mg/week) from hospital day 20. Inotropes were discontinued on hospital day 51 and her left ventricular ejection fraction had gradually improved to 37.5% at discharge (day 63). As AHF in TA is presumed to be due to inflammation of the myocardium, tocilizumab could be a treatment option for TA with AHF.