A Case of Takayasu's Artitis Which Presented as Total Occlusion of Right Pulmonary Artery

Abstract

A 59-year-old female with prior history of asthma and ulcerative colitis (UC) visited a clinic with a complaint of shortness of breath. She had treated with bronchodilator, but didn't improve. CT scan revealed total occlusion of right pulmonary artery (PA), so she was referred and admitted to our hospital for the further evaluation.Her vital signs were normal at rest, but SpO2 declined from 98% to 88% during 6-minute walk distance test. Laboratory data showed modest elevation of CRP (2.6mg/dL) and ESR. There was no evidence of pulmonary embolism.Ga scintigraphy showed accumulation in the hilum of the right lung, and PET-CT showed accumulation in the ascending aorta. Right heart catheterization revealed a mean pulmonary arterial pressure of 24 mmHg. Since she had cytoplasmic ANCA, history of asthma, and scarring of skin, we first suspected of ANCA-related vasculitis. However, skin and colon biopsy showed no typical findings. After excluding the possibility of any other vasculitis or viral infection, and considering HLA-B52 positive, we finally diagnosed as Takayasu's arteritis. ANCA sometimes be positive in UC, and since this patient had several clues to suspect ANCA-related vasculitis, it was challenging to give final diagnosis. Here, we report a case of Takayasu's arteritis which presented as total occlusion of right PA with borderline pulmonary hypertension. A 59-year-old female with prior history of asthma and ulcerative colitis (UC) visited a clinic with a complaint of shortness of breath. She had treated with bronchodilator, but didn't improve. CT scan revealed total occlusion of right pulmonary artery (PA), so she was referred and admitted to our hospital for the further evaluation. Her vital signs were normal at rest, but SpO2 declined from 98% to 88% during 6-minute walk distance test. Laboratory data showed modest elevation of CRP (2.6mg/dL) and ESR. There was no evidence of pulmonary embolism. Ga scintigraphy showed accumulation in the hilum of the right lung, and PET-CT showed accumulation in the ascending aorta. Right heart catheterization revealed a mean pulmonary arterial pressure of 24 mmHg. Since she had cytoplasmic ANCA, history of asthma, and scarring of skin, we first suspected of ANCA-related vasculitis. However, skin and colon biopsy showed no typical findings. After excluding the possibility of any other vasculitis or viral infection, and considering HLA-B52 positive, we finally diagnosed as Takayasu's arteritis. ANCA sometimes be positive in UC, and since this patient had several clues to suspect ANCA-related vasculitis, it was challenging to give final diagnosis. Here, we report a case of Takayasu's arteritis which presented as total occlusion of right PA with borderline pulmonary hypertension.