Postpartum onset Takayasu's arteritis presenting with aortic dissection.

Abstract

Takayasu's arteritis (TA), also known as pulseless disease and young female arteritis, is a chronic inflammatory large-vessel vasculitis (LVV). TA is pathologically characterized by arterial wall thickening, stenotic/occlusive lesions, aneurysm formation, and dissection. TA usually develops between 20 and 30 years of age. However, pregnancy and puerperium can affect the immune system, and several cases of postpartum onset or flare-up of TA have been reported. Herein, we report an extremely rare case of postpartum-onset TA complicated by aortic dissection. This is a case of Postpartum onset Takayasu's arteritis presenting with aortic dissection. A 34-year-old healthy woman was performed cesarean section. After 2 weeks, she presented with chest pain and fever, followed by mild dysphagia and hoarseness. Laboratory findings showed C-reactive protein (CRP) 21.61 mg/dl and computed tomography (CT) demonstrated thickening of the vessel wall of mainly ascending aorta. 18F-fluorodeoxyglucose (FDG)-position emission tomography (PET)/CT revealed high FDG uptake in the same areas. We diagnosed with TA and steroid pulse therapy was started. However, five days after treatment, the patient developed worsening symptoms of hoarseness. A contrast-enhanced CT showed Stanford A type dissection, and emergency artificial vessel replacement was performed. The specimen from surgical resection of the ascending aorta suggested active TA associated with dissection. The prednisolone dosage was gradually tapered with tocilizumab. Then, her symptoms and laboratory findings improved. It is important to recall the onset of TA and/or arterial dissection, when patients develop chest pain and hoarseness in the postpartum period.