肺高血圧精査にて診断しえたCREST症候群(限局性全身性硬化症)の1例

Pulmonary Hypertension Associated with CREST Syndrome

要旨　患者は78歳，女性．労作時呼吸困難を主訴に受診．動脈血ガス分析においてpO262mmHg，pCO231mmHgと低酸素血症を，心臓超音波検査では心室中隔の左室側への圧排像および三尖弁最大圧較差60mmHgと肺高血圧を認めた．胸部X線写真・CT上は低酸素血症を来す肺野病変を認めず，肺換気血流シンチグラフィでは換気血流ミスマッチを認めなかった．心臓カテーテル検査では冠動脈・左室造影に問題なく，O2step upも認めなかった．手指硬化性病変，Raynoud症状，食道病変，皮下石灰化像，毛細血管拡張，抗セントロメア抗体陽性からCREST症候群に伴う肺高血圧の診断に至り，Ca拮抗薬，抗凝固療法，酸素療法に加え経口プロスタグランディンI2(PGI2)誘導体の投与を開始し，その薬効評価を行った．PGI2誘導体の投与で酸素飽和度の上昇，肺動脈圧および総肺血管抵抗係数の著明な低下があり，急性効果が認められ有効と判断された．

Summary

　A 78-year-old female was admitted to our hospital due to the progressive dyspnea on effort. Blood gases analysis revealed hypoxia and echocardiogram showed that ventricular septum was oppressed to the left ventricle and the estimated systolic pressure of pulmonary artery was up to 60 mmHg. An X ray and computed tomography of chest showed no evidence of hypoxic state of lung parenchyma. A lung ventilation/perfusion scintigraphy did not show mis-match of ventilation/perfusion and heart catheterization did not find any shunt diseases. Her physical examination revealed calcinosis, Raynaud's phenomenon, esophageal motility disorders and telangiectasia. Serological examination revealed that anti-centromere antibody was positive. She was diagnosed as having moderate pulmonary hypertension associated with CREST syndrome. She was treated with calcium channel blocker, anticoagulant, oxygen therapy and started to receive oral PGI2 analogue. After about half a day of the treatment with oral PGI2, the systolic pulmonary artery pressure and total pulmonary vascular resistance index were significantly declined. Here we have shown that oral PGI2 acutely improves hemodynamics of the pulmonary hypertensive patient with CREST syndrome.