About this Journal Submit a Manuscript Table of Contents
Clinical and Developmental Immunology
Volume 2012 (2012), Article ID 854941, 12 pages
http://dx.doi.org/10.1155/2012/854941
Review Article

Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: Current Status and Future Direction

1Department of Medicine, North Bronx Healthcare Network, Jacobi Medical Center and North Central Bronx Hospital, 3424 Kossuth Avenue, Room 9C-01, Bronx, NY 10467, USA
2Department of Medicine, Albert Einstein College of Medicine, 1300 Morris Park Avenue, Bronx, NY 10461, USA

Received 9 November 2011; Accepted 2 January 2012

Academic Editor: Chaim Putterman

Copyright © 2012 Atiya Dhala. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Linked References

  1. V. V. McLaughlin, S. L. Archer, D. B. Badesch et al., “ACCF/AHA 2009 Expert Consensus Document on Pulmonary Hypertension. A Report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association Developed in Collaboration With the American College of Chest Physicians; American Thoracic Society, Inc.; and the Pulmonary Hypertension Association,” Journal of the American College of Cardiology, vol. 53, no. 17, pp. 1573–1619, 2009, Circulation, vol. 119, no. 16, pp. 2250–2294. View at Publisher · View at Google Scholar · View at PubMed
  2. P. M. Hassoun, “Pulmonary arterial hypertension complicating connective tissue diseases,” Seminars in Respiratory and Critical Care Medicine, vol. 30, no. 4, pp. 429–439, 2009. View at Publisher · View at Google Scholar · View at PubMed
  3. T. Yokoi, Y. Tomita, M. Fukaya, S. Ichihara, K. Kakudo, and Y. Takahashi, “Pulmonary hypertension associated with systemic lupus erythematosus: predominantly thrombotic arteriopathy accompanied by plexiform lesions,” Archives of Pathology and Laboratory Medicine, vol. 122, no. 5, pp. 467–470, 1998. View at Scopus
  4. H. T. Duong, S. C. Erzurum, and K. Asosingh, “Pro-angiogenic hematopoietic progenitor cells and endothelial colony-forming cells in pathological angiogenesis of bronchial and pulmonary circulation,” Angiogenesis, vol. 14, no. 4, pp. 411–422, 2011. View at Publisher · View at Google Scholar · View at PubMed
  5. R. Condliffe, D. G. Kiely, A. J. Peacock et al., “Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era,” American Journal of Respiratory and Critical Care Medicine, vol. 179, no. 2, pp. 151–157, 2009. View at Publisher · View at Google Scholar · View at PubMed
  6. L. Chung, J. Liu, L. Parsons et al., “Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL: identifying systemic sclerosis as a unique phenotype,” Chest, vol. 138, no. 6, pp. 1383–1394, 2010. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  7. M. Humbert, O. Sitbon, A. Chaouat et al., “Pulmonary arterial hypertension in France: results from a national registry,” American Journal of Respiratory and Critical Care Medicine, vol. 173, no. 9, pp. 1023–1030, 2006. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  8. A. J. Peacock, N. F. Murphy, J. J.V. McMurrey, L. Caballero, and S. Stewart, “An epidemiological study of pulmonary arterial hypertension,” European Respiratory Journal, vol. 30, no. 1, pp. 104–109, 2007. View at Publisher · View at Google Scholar · View at PubMed
  9. A. Prabu, K. Patel, C. S. Yee et al., “Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus,” Rheumatology, vol. 48, no. 12, pp. 1506–1511, 2009. View at Publisher · View at Google Scholar · View at PubMed
  10. J. S. Simonson, N. B. Schiller, M. Petri, and D. B. Hellmann, “Pulmonary hypertension in systemic lupus erythematosus,” Journal of Rheumatology, vol. 16, no. 7, pp. 918–925, 1989. View at Scopus
  11. T. M. Winslow, M. A. Ossipov, G. P. Fazio, J. S. Simonson, R. F. Redberg, and N. B. Schiller, “Five-year follow-up study of the prevalence and progression of pulmonary hypertension in systemic lupus erythematosus,” American Heart Journal, vol. 129, no. 3, pp. 510–515, 1995. View at Publisher · View at Google Scholar · View at Scopus
  12. F. P. Quismorio Jr., O. Sharma, M. Koss, et al., “Immunopathologic and clinical studies in pulmonary hypertension associated with systemic lupus erythematosus,” Seminars in Arthritis and Rheumatism, vol. 13, no. 4, pp. 349–359, 1984. View at Publisher · View at Google Scholar
  13. C. Haas, “Pulmonary hypertension associated with systemic lupus erythematosus,” Bulletin de l'Académie Nationale de Médecine, vol. 188, no. 6, pp. 985–997, 2004 (French).
  14. L. Arnaud, C. Agard, J. Haroche, P. Cacoub, J. C. Piette, and Z. Amoura, “Pulmonary arterial hypertension in systemic lupus erythematosus,” Revue de Medecine Interne, vol. 32, no. 11, pp. 689–697, 2011 (French). View at Publisher · View at Google Scholar · View at PubMed
  15. R. A. Asherson, T. W. Higenbottam, A. T. Dinh Xuan, M. A. Khamashta, and G. R. V. Hughes, “Pulmonary hypertension in a lupus clinic: experience with twenty-four patients,” Journal of Rheumatology, vol. 17, no. 10, pp. 1292–1298, 1990. View at Scopus
  16. R. A. Asherson, C. G. Mackworth-Young, M. L. Boey, et al., “Pulmonary hypertension in systemic lupus erythematosus,” British Medical Journal, vol. 287, no. 6398, pp. 1024–1025, 1983.
  17. A. Kasparian, A. Floros, E. Gialafos et al., “Raynaud's phenomenon is correlated with elevated systolic pulmonary arterial pressure in patients with systemic lupus erythematosus,” Lupus, vol. 16, no. 7, pp. 505–508, 2007. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  18. T. L. T. Pan, J. Thumboo, and M. L. Boey, “Primary and secondary pulmonary hypertension in systemic lupus erythematosus,” Lupus, vol. 9, no. 5, pp. 338–342, 2000. View at Scopus
  19. S. Miyamichi-Yamamoto, Y. Fukumoto, K. Sugimura et al., “Intensive immunosuppressive therapy improves pulmonary hemodynamics and long-term prognosis in patients with pulmonary arterial hypertension associated with connective tissue disease,” Circulation Journal, vol. 75, no. 11, pp. 2668–2674, 2011. View at Publisher · View at Google Scholar
  20. X. Jais, D. Launay, A. Yaici et al., “Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases,” Arthritis and Rheumatism, vol. 58, no. 2, pp. 521–531, 2008. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  21. O. Sanchez, O. Sitbon, X. Jaïs, G. Simonneau, and M. Humbert, “Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension,” Chest, vol. 130, no. 1, pp. 182–189, 2006. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  22. D. B. Badesch, N. S. Hill, G. Burgess, et al., “Sildenafil for pulmonary arterial hypertension associated with connective tissue disease,” The Journal of Rheumatology, vol. 34, no. 12, pp. 2417–2422, 2007.
  23. N. Galiè, H. A. Ghofrani, A. Torbicki et al., “Sildenafil citrate therapy for pulmonary arterial hypertension,” The New England Journal of Medicine, vol. 353, no. 20, pp. 2148–2157, 2005. View at Publisher · View at Google Scholar · View at PubMed
  24. L. J. Rubin, D. B. Badesch, R. J. Barst et al., “Bosentan therapy for pulmonary arterial hypertension,” The New England Journal of Medicine, vol. 346, no. 12, pp. 896–903, 2002. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  25. R. J. Oudiz, R. J. Schilz, R. J. Barst et al., “Treprostinil, a prostacyclin analogue, in pulmonary arterial hypertension associated with connective tissue disease,” Chest, vol. 126, no. 2, pp. 420–427, 2004. View at Publisher · View at Google Scholar · View at PubMed
  26. H. Olschewski, G. Simonneau, N. Galiè et al., “Inhaled iloprost for severe pulmonary hypertension,” The New England Journal of Medicine, vol. 347, no. 5, pp. 322–329, 2002. View at Publisher · View at Google Scholar · View at PubMed
  27. I. M. Robbins, S. P. Gaine, R. Schilz, V. F. Tapson, L. J. Rubin, and J. E. Loyd, “Epoprostenol for treatment of pulmonary hypertension in patients with systemic lupus erythematosus,” Chest, vol. 117, no. 1, pp. 14–18, 2000. View at Scopus
  28. J. B. Orens, F. J. Martinez, and J. P. Lynch III, “Pleuropulmonary manifestations of systemic lupus erythematosus,” Rheumatic Disease Clinics of North America, vol. 20, no. 1, pp. 159–193, 1994. View at Scopus
  29. J. Fernandez-Alonso, T. Zulueta, J. R. Reyes-Ramirez, M. J. Castillo-Palma, and J. Sanchez-Roman, “Pulmonary capillary hemangiomatosis as cause of pulmonary hypertension in a young woman with systemic lupus erythematosus,” Journal of Rheumatology, vol. 26, no. 1, pp. 231–233, 1999. View at Scopus
  30. D. Woolf, M. D. Voigt, K. Jaskiewicz, and A. A. Kalla, “Pulmonary hypertension associated with non-cirrhotic portal hypertension in systemic lupus erythematosus,” Postgraduate Medical Journal, vol. 70, no. 819, pp. 41–43, 1994. View at Scopus
  31. L. S. De Clerck, P. P. Michielsen, M. R. Ramael et al., “Portal and pulmonary vessel thrombosis associated with systemic lupus erythematosus and anticardiolipin antibodies,” Journal of Rheumatology, vol. 18, no. 12, pp. 1919–1921, 1991. View at Scopus
  32. O. Hubscher, A. Eimon, B. Elsner, and R. M. Arana, “Fatal post-partum pulmonary vasculitis in systemic lupus erythematosus,” Clinical Rheumatology, vol. 3, no. 4, pp. 547–550, 1984. View at Publisher · View at Google Scholar · View at Scopus
  33. H. M. Haupt, G. W. Moore, and G. M. Hutchins, “The lung in systemic lupus erythematosus. Analysis of the pathologic changes in 120 patients,” American Journal of Medicine, vol. 71, no. 5, pp. 791–798, 1981. View at Scopus
  34. L. A. Rubin, A. Geran, T. H. Rose, and H. Cohen, “A fatal pulmonary complication of lupus in pregnancy,” Arthritis and Rheumatism, vol. 38, no. 5, pp. 710–714, 1995. View at Scopus
  35. B. Corrin, H. Spencer, M. Turner Warwick, S. J. Beales, and J. J. Hamblin, “Pulmonary veno occlusion. An immune complex disease?” Virchows Archiv A, vol. 364, no. 1, pp. 81–91, 1974.
  36. N. Sasaki, A. Kamataki, and T. Sawai, “A histopathological study of pulmonary hypertension in connective tissue disease,” Allergology International, vol. 60, no. 4, pp. 411–417, 2011. View at Publisher · View at Google Scholar · View at PubMed
  37. J. Y. Shen, S. L. Chen, Y. X. Wu, et al., “Pulmonary hypertension in systemic lupus erythematosus,” Rheumatology International, vol. 18, no. 4, pp. 147–151, 1999.
  38. T. Yoshio, J. I. Masuyama, N. Kohda et al., “Association of interleukin 6 release from endothelial cells and pulmonary hypertension in SLE,” Journal of Rheumatology, vol. 24, no. 3, pp. 489–495, 1997. View at Scopus
  39. D. L. Kamen and C. Strange, “Pulmonary Manifestations of Systemic Lupus Erythematosus,” Clinics in Chest Medicine, vol. 31, no. 3, pp. 479–488, 2010. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  40. M. R. Nicolls, R. Tamosiuniene, N. F. Voelkel, and M. R. Nicolls, “Inflammation in pulmonary hypertension: how immunobiology provides the missing link between these conditions. Advances in pulmonary hypertension,” Autumn, vol. 5, no. 3, pp. 26–29, 2006.
  41. L. Harel, R. Straussberg, H. Rudich, A. H. Cohen, and J. Amir, “Raynaud's phenomenon as a manifestation of parvovirus B19 infection: case reports and review of parvovirus B19 rheumatic and vasculitic syndromes,” Clinical Infectious Diseases, vol. 30, no. 3, pp. 500–503, 2000. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  42. M. Abu-Shakra, S. Sukenik, and D. Buskila, “Systemic sclerosis: another rheumatic disease associated with hepatitis C virus infection,” Clinical Rheumatology, vol. 19, no. 5, pp. 378–380, 2000. View at Publisher · View at Google Scholar · View at Scopus
  43. J. Morse, R. Barst, E. Horn, N. Cuervo, Z. Deng, and J. Knowles, “Pulmonary hypertension in scleroderma spectrum of disease: lack of bone morphogenetic protein receptor 2 mutations,” Journal of Rheumatology, vol. 29, no. 11, pp. 2379–2381, 2002. View at Scopus
  44. B. Corrin and A. G. Nicholson, Pathology of the Lungs, 3rd edition, 2011.
  45. J. E. Loyd, “Pulmonary arterial hypertension: insights from genetic studies,” Proceedings of the American Thoracic Society, vol. 8, no. 2, pp. 154–157, 2011. View at Publisher · View at Google Scholar · View at PubMed
  46. A. J. Roncoroni, C. Alvarez, and F. Molinas, “Plexogenic arteriopathy associated with pulmonary vasculitis in systemic lupus erythematosus,” Respiration, vol. 59, no. 1, pp. 52–56, 1992. View at Scopus
  47. M. H. A. Rustin, H. A. Bull, S. J. Machin, D. A. Isenberg, M. L. Snaith, and P. M. Dowd, “Effects of the lupus anticoagulant in patients with systemic lupus erythematosus on endothelial cell prostacyclin release and procoagulant activity,” Journal of Investigative Dermatology, vol. 90, no. 5, pp. 744–748, 1988.
  48. A. Cefle, M. Inanc, M. Sayarlioglu et al., “Pulmonary hypertension in systemic lupus erythematosus: relationship with antiphospholipid antibodies and severe disease outcome,” Rheumatology International, vol. 31, no. 2, pp. 183–189, 2011. View at Publisher · View at Google Scholar · View at PubMed
  49. M. R. Nicolls, L. Taraseviciene-Stewart, P. R. Rai, D. B. Badesch, and N. F. Voelkel, “Autoimmunity and pulmonary hypertension: a perspective,” European Respiratory Journal, vol. 26, no. 6, pp. 1110–1118, 2005. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  50. C. Mihai and J. W. C. Tervaert, “Anti-endothelial cell antibodies in systemic sclerosis,” Annals of the Rheumatic Diseases, vol. 69, no. 2, pp. 319–324, 2010. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  51. D. S. Domiciano, J. F. Carvalho, and Y. Shoenfeld, “Pathogenic role of anti-endothelial cell antibodies in autoimmune rheumatic diseases,” Lupus, vol. 18, no. 13, pp. 1233–1238, 2009. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  52. S. S. Pullamsetti, R. Savai, W. Janssen et al., “Inflammation, immunological reaction and role of infection in pulmonary hypertension,” Clinical Microbiology and Infection, vol. 17, no. 1, pp. 7–14, 2011. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  53. S. W. Jamieson, D. P. Kapelanski, N. Sakakibara et al., “Pulmonary endarterectomy: experience and lessons learned in 1,500 cases,” Annals of Thoracic Surgery, vol. 76, no. 5, pp. 1457–1464, 2003. View at Publisher · View at Google Scholar
  54. P. Dartevelle, E. Fadel, S. Mussot et al., “Chronic thromboembolic pulmonary hypertension,” European Respiratory Journal, vol. 23, no. 4, pp. 637–648, 2004. View at Publisher · View at Google Scholar · View at Scopus
  55. M. Maggiorini, A. Knoblauch, J. Schneider, and E. W. Russi, “Diffuse microvascular pulmonary thrombosis associated with primary antiphospholipid antibody syndrome,” European Respiratory Journal, vol. 10, no. 3, pp. 727–730, 1997. View at Scopus
  56. V. A. Nguyen, T. Gotwald, C. Prior, G. Obermoser, and N. Sepp, “Acute pulmonary edema, capillaritis and alveolar hemorrhage: pulmonary manifestations coexistent in antiphospholipid syndrome and systemic lupus erythematosus?” Lupus, vol. 14, no. 7, pp. 557–560, 2005. View at Publisher · View at Google Scholar · View at Scopus
  57. R. A. Asherson, D. Hackett, and A. E. Gharavi, “Pulmonary hypertension in systemic lupus erythematosus: a report of three cases,” Journal of Rheumatology, vol. 13, no. 2, pp. 416–420, 1986.
  58. C. G. Mackworth-Young, A. E. Gharavi, M. L. Boey, and G. R. V. Hughes, “Portal and pulmonary hypertension in a case of systematic lupus erythematosus: possible relationship with a clotting abnormality,” European Journal of Rheumatology and Inflammation, vol. 7, no. 3, pp. 71–74, 1984. View at Scopus
  59. M. Miyata, K. Suzuki, F. Sakuma et al., “Anticardiolipin antibodies are associated with pulmonary hypertension in patients with mixed connective tissue disease or systemic lupus erythematosus,” International Archives of Allergy and Immunology, vol. 100, no. 4, pp. 351–354, 1993. View at Scopus
  60. P. A. Merkel, Y. Chang, S. S. Pierangeli, K. Convery, E. N. Harris, and R. P. Polisson, “The prevalence and clinical associations of anticardiolipin antibodies in a large inception cohort of patients with connective tissue diseases,” American Journal of Medicine, vol. 101, no. 6, pp. 576–583, 1996. View at Publisher · View at Google Scholar · View at Scopus
  61. N. Assous, Y. Allanore, F. Batteux et al., “Prevalence of antiphospholipid antibodies in systemic sclerosis and association with primitive pulmonary arterial hypertension and endothelial injury,” Clinical and Experimental Rheumatology, vol. 23, no. 2, pp. 199–204, 2005. View at Scopus
  62. G. Ruiz-Irastorza, M. Crowther, W. Branch, and M. A. Khamashta, “Antiphospholipid syndrome,” The Lancet, vol. 376, no. 9751, pp. 1498–1509, 2010. View at Publisher · View at Google Scholar · View at Scopus
  63. J. C. Crispin, A. Martínez, and J. Alcocer-Varela, “Quantification of regulatory T cells in patients with systemic lupus erythematosus,” Journal of Autoimmunity, vol. 21, no. 3, pp. 273–276, 2003. View at Publisher · View at Google Scholar · View at Scopus
  64. M. F. Liu, C. R. Wang, L. L. Fung, and C. R. Wu, “Decreased CD4+CD25+ T cells in peripheral blood of patients with systemic lupus erythematosus,” Scandinavian Journal of Immunology, vol. 59, no. 2, pp. 198–202, 2004. View at Publisher · View at Google Scholar
  65. F. Lian, D. Chen, Y. Wang et al., “Clinical features and independent predictors of pulmonary arterial hypertension in systemic lupus erythematosus,” Rheumatology International. In press.
  66. Y. F. Luo, I. M. Robbins, M. Karatas, A. G. Brixey, T. W. Rice, and R. W. Light, “Frequency of pleural effusions in patients with pulmonary arterial hypertension associated with connective tissue diseases,” Chest, vol. 140, no. 1, pp. 42–47, 2011. View at Publisher · View at Google Scholar · View at PubMed
  67. S. M. Arcasoy, J. D. Christie, V. A. Ferrari et al., “Echocardiographic assessment of pulmonary hypertension in patients with advanced lung disease,” American Journal of Respiratory and Critical Care Medicine, vol. 167, no. 5, pp. 735–740, 2003. View at Publisher · View at Google Scholar · View at PubMed
  68. H. W. Farber, A. J. Foreman, D. P. Miller, and M. D. Mcgoon, “REVEAL Registry: correlation of right heart catheterization and echocardiography in patients with pulmonary arterial hypertension,” Congestive Heart Failure, vol. 17, no. 2, pp. 56–64, 2011. View at Publisher · View at Google Scholar · View at PubMed
  69. J. D. Rich, S. J. Shah, R. S. Swamy, A. Kamp, and S. Rich, “Inaccuracy of doppler echocardiographic estimates of pulmonary artery pressures in patients with pulmonary hypertension: implications for clinical practice,” Chest, vol. 139, no. 5, pp. 988–993, 2011. View at Publisher · View at Google Scholar · View at PubMed
  70. P. Hodson, P. Klemp, and O. L. Meyers, “Pulmonary hypertension in systemic lupus erythematosus: a report of four cases,” Clinical and Experimental Rheumatology, vol. 1, no. 3, pp. 241–245, 1983. View at Scopus
  71. M. M. Hoeper, E. Mayer, G. Simonneau, and L. J. Rubin, “Chronic thromboembolic pulmonary hypertension,” Circulation, vol. 113, no. 16, pp. 2011–2020, 2006. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  72. I. M. Lang, “Chronic thromboembolic pulmonary hypertension—not so rare after all,” The New England Journal of Medicine, vol. 350, no. 22, pp. 2236–2238, 2004. View at Publisher · View at Google Scholar · View at PubMed
  73. M. Wolf, C. Boyer-Neumann, F. Parent et al., “Thrombotic risk factors in pulmonary hypertension,” European Respiratory Journal, vol. 15, no. 2, pp. 395–399, 2000. View at Publisher · View at Google Scholar · View at Scopus
  74. I. M. Lang, W. Klepetko, and I. Pabinger, “No increased prevalence of the factor V Leiden mutation in chronic major vessel thromboembolic pulmonary hypertension (CTEPH),” Thrombosis and haemostasis, vol. 76, no. 3, pp. 476–477, 1996.
  75. R. S. Kasthuri and R. A. S. Roubey, “Warfarin and the antiphospholipid syndrome: does one size fit all?” Arthritis Care and Research, vol. 57, no. 8, pp. 1346–1347, 2007. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  76. W. Klepetko, E. Mayer, J. Sandoval et al., “Interventional and surgical modalities of treatment for pulmonary arterial hypertension,” Journal of the American College of Cardiology, vol. 43, no. 12, supplement, pp. 73S–80S, 2004. View at Publisher · View at Google Scholar · View at PubMed
  77. N. Espínola-Zavaleta, J. Vargas-Barrón, T. Colmenares-Galvis et al., “Echocardiographic evaluation of patients with primary antiphospholipid syndrome,” American Heart Journal, vol. 137, no. 5, pp. 973–978, 1999. View at Publisher · View at Google Scholar · View at Scopus
  78. M. Turiel, P. Sarzi-Puttini, R. Peretti et al., “Five-year follow-up by transesophageal echocardiographic studies in primary antiphospholipid syndrome,” American Journal of Cardiology, vol. 96, no. 4, pp. 574–579, 2005. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  79. N. E. Zavaleta, R. M. Montes, M. E. Soto, N. A. Vanzzini, and M. C. Amigo, “Primary antiphospholipid syndrome: a 5-year transesophageal echocardiographic followup study,” Journal of Rheumatology, vol. 31, no. 12, pp. 2402–2407, 2004. View at Scopus
  80. G. Nesher, J. Ilany, D. Rosenmann, and A. S. Abraham, “Valvular dysfunction in antiphospholipid syndrome: prevalence, clinical features, and treatment,” Seminars in Arthritis and Rheumatism, vol. 27, no. 1, pp. 27–35, 1997. View at Publisher · View at Google Scholar
  81. D. M. Shahian, S. B. Labib, and A. B. Schneebaum, “Etiology and management of chronic valve disease in antiphospholipid antibody syndrome and systemic lupus erythematosus,” Journal of Cardiac Surgery, vol. 10, no. 2, pp. 133–139, 1995. View at Scopus
  82. M. Kato, H. Kataoka, T. Odani et al., “The short-term role of corticosteroid therapy for pulmonary arterial hypertension associated with connective tissue diseases: report of five cases and a literature review,” Lupus, vol. 20, no. 10, pp. 1047–1056, 2011. View at Publisher · View at Google Scholar · View at PubMed
  83. R. L. Benza, M. Gomberg-Maitland, R. Naeije, C. P. Arneson, and I. M. Lang, “Prognostic factors associated with increased survival in patients with pulmonary arterial hypertension treated with subcutaneous treprostinil in randomized, placebo-controlled trials,” Journal of Heart and Lung Transplantation, vol. 30, no. 9, pp. 982–989, 2011. View at Publisher · View at Google Scholar · View at PubMed
  84. S. Hennigan, R. N. Channick, and G. J. Silverman, “Rituximab treatment of pulmonary arterial hypertension associated with systemic lupus erythematosus: a case report,” Lupus, vol. 17, no. 8, pp. 754–756, 2008. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  85. R. D. Levy, A. J. Guerraty, M. H. Yacoub, and R. Loertscher, “Prolonged survival after heart-lung transplantation in systemic lupus erythematosus,” Chest, vol. 104, no. 6, pp. 1903–1905, 1993. View at Scopus
  86. E. B. Schwarz and S. Manzi, “Risk of unintended pregnancy among women with systemic lupus erythematosus,” Arthritis Care and Research, vol. 59, no. 6, pp. 863–866, 2008. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  87. M. A. Greenstone, “Delayed diagnosis of systemic lupus erythematosus associated pulmonary hypertension,” British Journal of Rheumatology, vol. 30, no. 5, p. 391, 1991. View at Scopus
  88. J. Ray and M. Sermer, “Systemic lupus erythematosus and pulmonary hypertension during pregnancy: report of a case fatality,” Canadian Journal of Cardiology, vol. 12, no. 8, pp. 753–756, 1996. View at Scopus
  89. W. L. Martin, C. Gordon, and M. D. Kilby, “Systemic lupus erythematosus,” The Lancet, vol. 358, no. 9281, p. 586, 2001. View at Scopus
  90. B. M. Weiss, L. Zemp, B. Seifert, and O. M. Hess, “Outcome of pulmonary vascular disease in pregnancy: a systematic overview from 1978 through 1996,” Journal of the American College of Cardiology, vol. 31, no. 7, pp. 1650–1657, 1998. View at Publisher · View at Google Scholar · View at Scopus
  91. S. A. Quadrelli, C. Alvarez, S. C. Arce et al., “Pulmonary involvement of systemic lupus erythematosus: analysis of 90 necropsies,” Lupus, vol. 18, no. 12, pp. 1053–1060, 2009. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  92. W. U. Kim, J. K. Min, S. H. Lee, S. H. Park, C. S. Cho, and H. Y. Kim, “Causes of death in Korean patients with systemic lupus erythematosus: a single center retrospective study,” Clinical and Experimental Rheumatology, vol. 17, no. 5, pp. 539–545, 1999. View at Scopus
  93. S. M. Chung, C. K. Lee, E. Y. Lee, B. Yoo, S. D. Lee, and H. B. Moon, “Clinical aspects of pulmonary hypertension in patients with systemic lupus erythematosus and in patients with idiopathic pulmonary arterial hypertension,” Clinical Rheumatology, vol. 25, no. 6, pp. 866–872, 2006. View at Publisher · View at Google Scholar · View at PubMed · View at Scopus
  94. R. Condliffe, D. G. Kiely, A. J. Peacock, et al., “Initial results of the UK audit of pulmonary arterial hypertension associated with connective tissue disease,” European Heart Journal, vol. 28, pp. 139–140, 2007.