抗シンテターゼ症候群と肺静脈閉塞性疾患を合併している

Anti-synthetase syndrome-associated pulmonary veno-occlusive disease.

• Diana Kay
• Ferdous Kadri
• Garrett Fitzpatrick
• Hassan Alnuaimat
• Raju Reddy
• Ali Ataya

抄録

肺動脈性高血圧症の有病率は7.9％と報告されているが，抗シンテターゼ症候群と肺静脈閉塞性疾患（PVOD）を合併した症例はこれまでに報告されたことがない．本研究では，右心不全を伴う低酸素性呼吸不全を呈し，自己免疫血清検査と右心カテーテル検査での毛細血管前肺高血圧から抗シンテターゼ症候群と診断された患者を対象に，抗シンテターゼ症候群と肺静脈閉塞症を合併した新しい症例を紹介する．肺動脈性肺高血圧症治療を開始したが，非経口エポプロステノールの増量に伴い，胸部画像検査でPVODを疑う急性増悪を認め，剖検で確認された．抗シンテターゼ症候群はPVODと関連している可能性があり，前毛細血管性肺高血圧症の既往歴があり，肺高血圧症特異的治療の開始に伴って悪化した患者では疑うべきである．

Pulmonary arterial hypertension has been reported with a prevalence of 7.9% in patients with anti-synthetase syndrome; however, anti-synthetase syndrome associated with pulmonary veno-occlusive disease (PVOD) has never before been described in the literature. We present a novel case of anti-synthetase syndrome-associated PVOD in a patient who presented with hypoxic respiratory failure associated with right heart failure and was diagnosed with anti-synthetase syndrome based on his autoimmune serology and pre-capillary pulmonary hypertension on right heart catheterization. He was initiated on pulmonary arterial hypertension therapy, but with escalating dose of parenteral epoprostenol, experienced acute clinical worsening with chest imaging concerning for PVOD that was confirmed on autopsy. Anti-synthetase syndrome can be associated with PVOD, and it should be suspected in patients who have evidence of pre-capillary pulmonary hypertension and who deteriorate with the initiation of pulmonary hypertension-specific therapy.

© The Author(s) 2020.