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

Background We encountered a 12-year-old girl with Takayasu arteritis (TA) who developed acute heart failure at onset. There have been few reports of specific treatment for acute heart failure (AHF) in TA. We successfully treated her with intravenous methylprednisolone and tocilizumab. Case presentation The patient developed palpitations and shortness of breath 3 weeks before admission. Her symptoms exacerbated rapidly and she finally entered our hospital intensive care unit due to respiratory distress and tachycardia. Blood pressure values measured on the left arm and bilateral legs were paradoxically lower than values taken 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 (LVEF) was 20% on cardiac ultrasound. Laboratory examination suggested acute inflammation and positivity of HLA-B52. Her medical condition was finally diagnosed as TA with AHF. Along with inotropes and diuretics, methylprednisolone pulse therapy was administered for 3 days on the 2nd and 12th hospital day followed by oral prednisolone. Cardiac function was slightly improved. As intravenous cyclophosphamide therapy requires hydration and may exacerbate AHF, we started weekly subcutaneous tocilizumab treatment (162 mg/week) from the 20th hospital day. Inotropes were discontinued on the 51st hospital day and her LVEF had improved to 37.5% on the 63rd day when she was discharged. Conclusions Tocilizumab could be a significant treatment option for acute heart failure in juvenile TA.