Thromb Haemost 2019; 119(09): 1403-1408
DOI: 10.1055/s-0039-1692428
Coagulation and Fibrinolysis
Georg Thieme Verlag KG Stuttgart · New York

Antiphospholipid Syndrome in Chronic Thromboembolic Pulmonary Hypertension: A Well-Defined Subgroup of Patients

Xin Jiang*
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Yao Du*
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Chun-Yan Cheng
2   Cardiology Clinic, Thrombosis Centre, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy
,
Gentian Denas
2   Cardiology Clinic, Thrombosis Centre, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy
,
Yu-Ping Zhou
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Tao Wu
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Yi-Xin Zhang
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Zhi-Yan Han
3   Department of Anaesthesiology, FuWai Hospital, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
,
Vittorio Pengo
2   Cardiology Clinic, Thrombosis Centre, Department of Cardiac, Thoracic, Vascular Sciences and Public Health, University of Padua, Padua, Italy
,
1   FuWai Hospital & Key Laboratory of Pulmonary Vascular Medicine, State Key Laboratory of Cardiovascular Disease, National Centre for Cardiovascular Diseases, Peking Union Medical College, Chinese Academy Medical Sciences, Beijing, China
› Institutsangaben
Funding This study was supported by Capital Clinical Specialty Project of Beijing Municipal (Z181100001718203 and Z171100001017195). CAMS Innovation Fund for Medical Sciences (CIFMS) (2016-I2M-1–002, 2017-I2M-3–003, 2017-I2M-2–001, and 2017-I2M-B&R-02). National Science Fund for Distinguished Young Scholars (81425002). The National Key Research and Development Program of China (2016YFC0901502).
Weitere Informationen

Publikationsverlauf

20. Februar 2019

01. Mai 2019

Publikationsdatum:
21. Juni 2019 (online)

Abstract

Antiphospholipid syndrome (APS) is an acquired thrombophilia with an uncertain role in the development of chronic thromboembolic pulmonary hypertension (CTEPH). We aimed to assess the association of APS with the clinical phenotype of CTEPH. We retrospectively reviewed data of CTEPH patients referred to our center. Clinical, angiographic, and hemodynamic data were available for all patients. APS was diagnosed in the presence of one or more positive antiphospholipid (aPL) tests confirmed more than 12 weeks apart. Data were compared between APS-positive and APS-negative patients. From May 2013 to December 2018, 297 patients with CTEPH were enrolled. Twenty-three (7.7%) were positive for laboratory tests exploring aPL antibodies. Among them, 17 patients (74%) had a triple positive aPL profile. When compared with the APS-negative group, APS patients were significantly younger (30.0 ± 11.1 vs. 55.6 ± 12.9 years, p < 0.0001), had more frequently a history of pulmonary embolism (95.6% vs. 65.7%, p = 0.003), and had more frequently associated autoimmune disease (43.5% vs. 2.9%, p < 0.0001). In APS-positive patients, pulmonary artery lesions were more proximal and hemodynamic profiles were less compromised. Our results show that patients with APS are a unique group of CTEPH patients with well-defined clinic and hemodynamic characteristics.

* Xin Jiang and Yao Du equally contributed to this article.


 
  • References

  • 1 Kim NH, Delcroix M, Jais X. , et al. Chronic thromboembolic pulmonary hypertension. Eur Respir J 2019; 53 (01) 1801915
  • 2 Mahmud E, Madani MM, Kim NH. , et al. Chronic thromboembolic pulmonary hypertension: evolving therapeutic approaches for operable and inoperable disease. J Am Coll Cardiol 2018; 71 (21) 2468-2486
  • 3 Simonneau G, Torbicki A, Dorfmüller P, Kim N. The pathophysiology of chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26 (143) 160112
  • 4 Pengo V, Lensing AW, Prins MH. , et al; Thromboembolic Pulmonary Hypertension Study Group. Incidence of chronic thromboembolic pulmonary hypertension after pulmonary embolism. N Engl J Med 2004; 350 (22) 2257-2264
  • 5 Miyakis S, Lockshin MD, Atsumi T. , et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost 2006; 4 (02) 295-306
  • 6 Cervera R, Piette JC, Font J. , et al; Euro-Phospholipid Project Group. Antiphospholipid syndrome: clinical and immunologic manifestations and patterns of disease expression in a cohort of 1,000 patients. Arthritis Rheum 2002; 46 (04) 1019-1027
  • 7 Wong CL, Szydlo R, Gibbs S, Laffan M. Hereditary and acquired thrombotic risk factors for chronic thromboembolic pulmonary hypertension. Blood Coagul Fibrinolysis 2010; 21 (03) 201-206
  • 8 Pepke-Zaba J, Delcroix M, Lang I. , et al. Chronic thromboembolic pulmonary hypertension (CTEPH): results from an international prospective registry. Circulation 2011; 124 (18) 1973-1981
  • 9 Colorio CC, Martinuzzo ME, Forastiero RR, Pombo G, Adamczuk Y, Carreras LO. Thrombophilic factors in chronic thromboembolic pulmonary hypertension. Blood Coagul Fibrinolysis 2001; 12 (06) 427-432
  • 10 Cheng CY, Zhang YX, Denas G, Du Y, Jing ZC, Pengo V. Prevalence of antiphospholipid (aPL) antibodies among patients with chronic thromboembolic pulmonary hypertension: a systematic review and meta-analysis. Intern Emerg Med 2019; 14 (04) 521-527
  • 11 Pengo V, Ruffatti A, Legnani C. , et al. Clinical course of high-risk patients diagnosed with antiphospholipid syndrome. J Thromb Haemost 2010; 8 (02) 237-242
  • 12 Galiè N, Humbert M, Vachiery JL. , et al. 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Respir J 2015; 46 (04) 903-975
  • 13 Devreese KMJ, Ortel TL, Pengo V, de Laat B. ; Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibodies. Laboratory criteria for antiphospholipid syndrome: communication from the SSC of the ISTH. J Thromb Haemost 2018; 16 (04) 809-813
  • 14 Pengo V, Tripodi A, Reber G. , et al; Subcommittee on Lupus Anticoagulant/Antiphospholipid Antibody of the Scientific and Standardisation Committee of the International Society on Thrombosis and Haemostasis. Update of the guidelines for lupus anticoagulant detection. J Thromb Haemost 2009; 7 (10) 1737-1740
  • 15 Jenkins D, Madani M, Fadel E, D'Armini AM, Mayer E. Pulmonary endarterectomy in the management of chronic thromboembolic pulmonary hypertension. Eur Respir Rev 2017; 26 (143) 160111
  • 16 Ruffatti A, De Silvestro G, Marson P. , et al. Catastrophic antiphospholipid syndrome: Lessons from 14 cases successfully treated in a single center. A narrative report. J Autoimmun 2018; 93: 124-130
  • 17 Pengo V, Biasiolo A, Gresele P. , et al. A comparison of lupus anticoagulant-positive patients with clinical picture of antiphospholipid syndrome and those without. Arterioscler Thromb Vasc Biol 2007; 27 (12) e309-e310
  • 18 Gall H, Hoeper MM, Richter MJ, Cacheris W, Hinzmann B, Mayer E. An epidemiological analysis of the burden of chronic thromboembolic pulmonary hypertension in the USA, Europe and Japan. Eur Respir Rev 2017; 26 (143) 160121
  • 19 Morpurgo M, Schmid C. The spectrum of pulmonary embolism. Clinicopathologic correlations. Chest 1995; 107 (1, Suppl): 18S-20S