저자들은 폐출혈, 현미경적 혈뇨와 단백뇨, 말초 신경병증에 의한 사지의 운동 및 감각신경장애, 말초 혈관염에 의해 급속히 진행하는 손발끝의 건성 괴저 등의 임상양상을 보이는 66세 남자 환자를 현미경적 다발성 혈관염으로 진단하였다. 또한, 폐출혈이 멈추고, 흉부 방사선학적 소견이 경미하였는데도 갑자기 호흡부전으로 진행하여, 폐색전증으로 오인되었던 Swyer-James 증후군을 경험하였기에 문헌고찰과 함께 보고하는 바이다.
저자들은 폐출혈, 현미경적 혈뇨와 단백뇨, 말초 신경병증에 의한 사지의 운동 및 감각신경장애, 말초 혈관염에 의해 급속히 진행하는 손발끝의 건성 괴저 등의 임상양상을 보이는 66세 남자 환자를 현미경적 다발성 혈관염으로 진단하였다. 또한, 폐출혈이 멈추고, 흉부 방사선학적 소견이 경미하였는데도 갑자기 호흡부전으로 진행하여, 폐색전증으로 오인되었던 Swyer-James 증후군을 경험하였기에 문헌고찰과 함께 보고하는 바이다.
A 66-year-old man was admitted to our institution with a one-month history of benumbed extremities and a small amount of hemoptysis. Laboratory findings showed microscopic hematuria and proteinuria along with a positive P-ANCA test. The third day after admission, he developed dry gangrene and motor ...
A 66-year-old man was admitted to our institution with a one-month history of benumbed extremities and a small amount of hemoptysis. Laboratory findings showed microscopic hematuria and proteinuria along with a positive P-ANCA test. The third day after admission, he developed dry gangrene and motor weakness in the upper and lower extremities. Nerve biopsy demonstrated leukocytoclastic vasculitis. Microscopic polyangiitis was diagnosed based on clinical and pathological clues. Despite sufficient oxygen therapy, his respiratory status suddenly deteriorated, and the possibility of pulmonary thromboembolism was suggested. Pulmonary perfusion scintigraphy showed markedly decreased perfusion in the right lung. However, chest CT revealed hypoplasia of the left pulmonary artery and hyperlucency on the left lung, which are consistent with Swyer-James syndrome, a non-filling defect in the pulmonary arteries. There has been no report of a case of Swyer-James syndrome accompanied by MPA. We here report a case of Swyer-James syndrome mimicking pulmonary thromboembolism in a patient with microscopic polyangiitis.
A 66-year-old man was admitted to our institution with a one-month history of benumbed extremities and a small amount of hemoptysis. Laboratory findings showed microscopic hematuria and proteinuria along with a positive P-ANCA test. The third day after admission, he developed dry gangrene and motor weakness in the upper and lower extremities. Nerve biopsy demonstrated leukocytoclastic vasculitis. Microscopic polyangiitis was diagnosed based on clinical and pathological clues. Despite sufficient oxygen therapy, his respiratory status suddenly deteriorated, and the possibility of pulmonary thromboembolism was suggested. Pulmonary perfusion scintigraphy showed markedly decreased perfusion in the right lung. However, chest CT revealed hypoplasia of the left pulmonary artery and hyperlucency on the left lung, which are consistent with Swyer-James syndrome, a non-filling defect in the pulmonary arteries. There has been no report of a case of Swyer-James syndrome accompanied by MPA. We here report a case of Swyer-James syndrome mimicking pulmonary thromboembolism in a patient with microscopic polyangiitis.
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