A 12-year-old girl had developed palpitations and exertional dyspnea 3 weeks before admission. Subsequently, she presented with general fatigue, facial pallor, and orthopnea. AHF was suspected by her primary physician and she was transferred to our intensive care unit. She had no remarkable history of unknown fever suggesting TA. On admission, she required oxygen (FiO2: 0.60, high-flow nasal cannula) due to respiratory distress. Consciousness level was clear. Her heart rate was 150/min and her respiratory rate was 50/min. Her blood pressure was 100/60 mmHg on her right arm, but was paradoxically lower (80/50 mmHg) on her left arm and bilateral legs. Capillary refilling time was 2.0 s. Gallop sounds, bilateral moist rales, and coarse crackles were heard. The abdomen was soft and not distended.
Chest X-ray showed significant cardiomegaly, bilateral pulmonary congestion, and marked pleural effusion. Cardiac ultrasound revealed four chambers severely dilated, diffuse decrease of wall motion, moderate aortic regurgitation (AR), and mitral regurgitation (MR). The left ventricular ejection fraction (LVEF) was 20%. Contrast-enhanced computed tomography revealed severely dilated cardiac chambers, aneurysm of the ascending and descending aorta, alternating dilatation and stenosis of the abdominal aorta, and intraluminal thrombi (Figure 1). Laboratory investigation revealed cardiac failure, microcytic anemia, inflammatory findings, renal impairment, and hypergammaglobulinemia. White blood cell count was 9210/µL (51% neutrophils), hemoglobin 7.1 g/dL, mean corpuscular volume 61.5 fL, and mean corpuscular hemoglobin 17.0 pg. Erythrocyte sedimentation rate was 56 mm/hour and C-reactive protein 2.99 mg/dL. Blood urea nitrogen was 28.3 mg/dL, creatinine 0.59 mg/dL; and levels of aspartate aminotransferase, lactate dehydrogenase, and creatine kinase-muscle/brain were normal. Brain natriuretic peptide was 1879.9 pg/mL (reference range: 0.0-18.4 pg/mL) and troponin-T 50-100 ng/L (reference range: <14 ng/L). Serum IgG was 2277 mg/dL, IgA 432 mg/dL, IgM 169 mg/dL, complement component-3 103 mg/dL (reference range: 86-160 mg/dL), complement component-4 9 mg/dL (reference range: 11-31 mg/dL), 50% hemolytic complement 30.5 U/mL (reference range: 25.0-48.0 U/mL), and antinuclear antibodies were negative. We also subsequently found positivity of human leukocyte antigen-B52.
According to radiological findings and laboratory examinations, the patient’s disease was diagnosed as TA. The patient was immediately treated with inotropes, diuretics, intravenous heparin, and corticosteroids, including two courses of methylprednisolone pulse therapy (1000 mg/day) on the 2nd and 12th hospital day followed by 30 mg of daily oral prednisolone. However, her cardiac function did not notably improve (Figure 2). For remission induction and further improvement, IVCY was initially considered, but this would require aggressive intravenous hydration for prevention of hemorrhagic cystitis. Such volume overload might exacerbate AHF. We decided to use weekly tocilizumab. Subcutaneous tocilizumab was initiated on the 20th hospital day after obtaining informed consent from the guardian. Her cardiac function gradually recovered. BNP also decreased (Figure 2). Inotropes were discontinued on the 51st hospital day. At discharge on the 63rd hospital day, cardiac ultrasound still exhibited a dilated left ventricle, moderate AR, and trivial MR. However, LVEF had improved to 38% and mural thrombosis in the ascending aorta was reduced in size.
As of this writing it has been two years from onset. She has had no recurrence of TA. Cardiac ultrasound exhibited mild AR and LVEF had improved to 69%. Currently she is being treated with 6 mg of daily prednisolone, 1500 mg of daily mycophenolate mofetil, 12 mg of weekly methotrexate, and 162 mg of weekly subcutaneous tocilizumab. No severe adverse effects of tocilizumab have been observed.