항인지질 증후군과 전신성 홍반성 루푸스 환자에게 발생된 만성 폐혈전색전성 폐동맥 고혈압에 대해 시행한 혈전내막제거술

Pulmonary Thromboendarterectomy for Chronic Thromboembolic Pulmonary Hypertension in a Patient with Antiphospholipid Syndrome and Systemic Lupus Erythematosus

  • 강필제 (울산대학교 의과대학 서울아산병원 흉부외과학교실) ;
  • 김정원 (울산대학교 의과대학 울산대학교병원 흉부외과학교실) ;
  • 이재원 (울산대학교 의과대학 서울아산병원 흉부외과학교실)
  • Kang, Pil-Je (Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine) ;
  • Kim, Jeong-Won (Department of Thoracic and Cardiovascular Surgery, Ulsan University Hospital, University of Ulsan College of Medicine) ;
  • Lee, Jae-Won (Department of Thoracic and Cardiovascular Surgery, Asan Medical Center, University of Ulsan College of Medicine)
  • 발행 : 2007.12.05

초록

항인지질 증후군(antiphospholipid syndrome)은 정맥, 동맥의 혈전증, 혈소판 감소증, 반복적인 유산 등과 함께 lupus anticoagulant (LAC)와 anticardiolipin antibody (aCL)가 반복적으로 양성 반응을 보이는 질환을 말하며 호흡기 증후 발현은 상대적으로 드물다. 저자들은 항인지질 증후군 및 전신성 홍반성 루푸스 환자에서 발생한 만성적인 폐혈전색전증에 대하여 폐동맥 내막절제술, 특히 원위부에 있는 폐혈전색전증을 왼쪽 폐의 대열을 분리한 뒤 좌하엽 구역 동맥을 절제하여 혈전색전 제거술을 성공적으로 시행하였기에 문헌고찰과 함께 국내 최초로 보고하는 바이다.

Antiphospholipid syndrome (APS) is defined as venous and/or arterial thromboses, recurrent fetal losses, thrombocytopenia in combination with repeatedly positive tests for the lupus anticoagulant (LAC), and anticardiolipin antibodies (aCL). The pulmonary manifestation is APS are relatively rare. We report a rare case of antiphopholipid syndrome with systemic lupus erythematosus in a patient who presented with pulmonary hypertension secondary to a chronic pulmonary thromboembolism. A bilateral thromboendarterectomy was performed satisfactorily and the incision was extended to the left intrapleural pulmonary artery.

키워드

참고문헌

  1. Stojanovich L. Pulmonary manifestations in antiphospholipid syndrome. Autoimmun Rev 2006;5:344-8 https://doi.org/10.1016/j.autrev.2006.02.002
  2. Wilson WA, Gharavi AE, Koike T, et al. International consensus statement on preliminary classification criteria for definite antiphospholipid syndrome: report of an international workshop. Arthritis Rheum 1999;42:1309-11 https://doi.org/10.1002/1529-0131(199907)42:7<1309::AID-ANR1>3.0.CO;2-F
  3. Medina G, Vera-Lastra O, Barile L, Salas M, Jara LJ. Clinical spectrum of males with primary antiphospholipid syndrome and systemic lupus erythematosus: a comparative study of 73 patients. Lupus 2004;13:11-6 https://doi.org/10.1191/0961203304lu482oa
  4. Nakajima T, Ando H, Ueno Y, et al. Successful thromboendarterectomy for chronic pulmonary embolism in a patient with systemic lupus erythematosus and antiphospholipid syndrome. Jpn Circ J 1997;61:958-64 https://doi.org/10.1253/jcj.61.958
  5. Rosove MH, Brewer PM. Antiphospholipid thrombosis: clinical course after the first thrombotic event in 70 patients. Ann Intern Med 1992;117:303-8 https://doi.org/10.7326/0003-4819-117-4-303
  6. Song SH, Park PW, Jun TG, et al, Pulmonary thromboendarterectomy for pulmonary hypertension caused by chronic pulmonary thromboembolism. Korean J Thorac Cardiocasc Surg 2006;39:626-32
  7. Cooley DA, Beall AC Jr, Alexander JK. Acute massive pulmonary embolism. Successful surgical treatment using temporary cardiopulmonary bypass. JAMA 1961;177:283-6 https://doi.org/10.1001/jama.1961.03040310001001
  8. Thistlethwaite PA, Madani M, Jamieson SW. Outcomes of pulmonary endarterectomy surgery. Semin Thorac Cardiovasc Surg 2006;18:257-64 https://doi.org/10.1053/j.semtcvs.2006.09.008