Tohoku J. Exp. Med., 2018 December, 246(4), 251-256.

Onset of Takotsubo Syndrome during the Clinical Course of Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis: A Case Report


1Department of Nephrology, Nagano Red Cross Hospital, Nagano, Nagano, Japan
2Department of Nephrology, Shinshu University School of Medicine, Matsumoto, Nagano, Japan

Anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a systemic vasculitis resulting in severe organ injuries. ANCA is a disease-labeled antibody of AAV, and myeloperoxidase (MPO) and proteinase 3 are the main targeted antigens of ANCA. Takotsubo syndrome, a transient cardiac dysfunction caused by emotional or physical stress, is characterized by ST-segment elevation and negative T waves in electrocardiogram, transient left ventricular asynergy, and absence of obstructive coronary disease. To the best of our knowledge, only two cases of coexistence of AAV and takotsubo syndrome have been reported. Herein, we report the case of AAV complicated with takotsubo syndrome. A 78-year-old Japanese woman presented with severe renal dysfunction, which was diagnosed as MPO-ANCA-associated systemic vasculitis. Despite the treatment with cyclophosphamide and glucocorticoid, the patient presented with severe respiratory failure due to alveolar hemorrhage and heart failure. Electrocardiography indicated newly developed T wave inversions. Echocardiography demonstrated severe left ventricular dysfunction with hypokinesis of the apical area. Moreover, coronary angiography revealed no noticeable stenotic or obstructive lesions. These findings indicate the onset of takotsubo syndrome. After immunosuppressive therapy, systemic vasculitis and takotsubo syndrome were improved. Although a coexisting case of AAV and takotsubo syndrome is rare, we have to consider the possible complication of takotsubo syndrome in case of presenting acute heart failure. Considering the present case and the previously reported coexisting cases of takotsubo syndrome and AAV, we propose that female sex, initiation of glucocorticoid therapy, and high titer of MPO-ANCA are potential risk factors of developing takotsubo syndrome.

Key words —— alveolar hemorrhage; anti-neutrophil cytoplasmic antibody; anti-neutrophil cytoplasmic antibody-associated vasculitis; heart failure; takotsubo cardiomyopathy


Tohoku J. Exp. Med., 2018, 246, 251-256

Correspondence: Makoto Harada, M.D., Ph.D., Department of Nephrology, Nagano Red Cross Hospital, 5-22-1 Wakasato, Nagano, Nagano 380-8582, Japan.