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Volumn 8, Issue 3, 2012, Pages 413-425

Pulmonary Arterial Hypertension in Connective Tissue Diseases

Author keywords

Connective tissue disease; Mixed connective tissue disease; Pulmonary hypertension; Scleroderma; Systemic lupus erythematosus; Therapy

Indexed keywords

ACTIVIN RECEPTOR LIKE KINASE 1; AMBRISENTAN; AMINO TERMINAL PRO BRAIN NATRIURETIC PEPTIDE; AUTOANTIBODY; BONE MORPHOGENETIC PROTEIN RECEPTOR 2; BOSENTAN; CD31 ANTIGEN; CELL ADHESION MOLECULE; CORTICOSTEROID; ENDOGLIN; HLA B35 ANTIGEN; IMATINIB; IMMUNOSUPPRESSIVE AGENT; INTERCELLULAR ADHESION MOLECULE 1; INTERLEUKIN 10; INTERLEUKIN 1ALPHA; MONOCYTE CHEMOTACTIC PROTEIN 1; PROSTACYCLIN; PROSTAGLANDIN; PROSTAGLANDIN DERIVATIVE; SILDENAFIL; SITAXSENTAN; TRANSFORMING GROWTH FACTOR BETA; TRANSFORMING GROWTH FACTOR BETA RECEPTOR; UNIPROST; VASCULAR CELL ADHESION MOLECULE 1; VASCULOTROPIN;

EID: 84863084082     PISSN: 15517136     EISSN: None     Source Type: Journal    
DOI: 10.1016/j.hfc.2012.04.001     Document Type: Review
Times cited : (51)

References (126)
  • 1
    • 0025837793 scopus 로고
    • Survival in patients with primary pulmonary hypertension. Results from a national prospective registry
    • D'Alonzo G.E., Barst R.J., Ayres S.M., et al. Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 1991, 115:343-349.
    • (1991) Ann Intern Med , vol.115 , pp. 343-349
    • D'Alonzo, G.E.1    Barst, R.J.2    Ayres, S.M.3
  • 2
    • 0032578279 scopus 로고    scopus 로고
    • Primary pulmonary hypertension
    • Gaine S.P., Rubin L.J. Primary pulmonary hypertension. Lancet 1998, 352:719-725.
    • (1998) Lancet , vol.352 , pp. 719-725
    • Gaine, S.P.1    Rubin, L.J.2
  • 3
    • 0023615359 scopus 로고
    • Primary pulmonary hypertension. A national prospective study
    • Rich S., Dantzker D.R., Ayres S.M., et al. Primary pulmonary hypertension. A national prospective study. Ann Intern Med 1987, 107:216-223.
    • (1987) Ann Intern Med , vol.107 , pp. 216-223
    • Rich, S.1    Dantzker, D.R.2    Ayres, S.M.3
  • 4
    • 67649579669 scopus 로고    scopus 로고
    • Updated clinical classification of pulmonary hypertension
    • Simonneau G., Robbins I.M., Beghetti M., et al. Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 2009, 54:S43-S54.
    • (2009) J Am Coll Cardiol , vol.54
    • Simonneau, G.1    Robbins, I.M.2    Beghetti, M.3
  • 5
    • 1642453778 scopus 로고    scopus 로고
    • Endothelial dysfunction in pulmonary hypertension
    • Budhiraja R., Tuder R.M., Hassoun P.M. Endothelial dysfunction in pulmonary hypertension. Circulation 2004, 109:159-165.
    • (2004) Circulation , vol.109 , pp. 159-165
    • Budhiraja, R.1    Tuder, R.M.2    Hassoun, P.M.3
  • 6
    • 4644290657 scopus 로고    scopus 로고
    • Treatment of pulmonary arterial hypertension
    • Humbert M., Sitbon O., Simonneau G. Treatment of pulmonary arterial hypertension. N Engl J Med 2004, 351:1425-1436.
    • (2004) N Engl J Med , vol.351 , pp. 1425-1436
    • Humbert, M.1    Sitbon, O.2    Simonneau, G.3
  • 7
    • 0026754440 scopus 로고
    • Autoantibodies in patients with primary pulmonary hypertension: association with anti-Ku
    • Isern R.A., Yaneva M., Weiner E., et al. Autoantibodies in patients with primary pulmonary hypertension: association with anti-Ku. Am J Med 1992, 93:307-312.
    • (1992) Am J Med , vol.93 , pp. 307-312
    • Isern, R.A.1    Yaneva, M.2    Weiner, E.3
  • 8
    • 0029062419 scopus 로고
    • Increased interleukin-1 and interleukin-6 serum concentrations in severe primary pulmonary hypertension
    • Humbert M., Monti G., Brenot F., et al. Increased interleukin-1 and interleukin-6 serum concentrations in severe primary pulmonary hypertension. Am J Respir Crit Care Med 1995, 151:1628-1631.
    • (1995) Am J Respir Crit Care Med , vol.151 , pp. 1628-1631
    • Humbert, M.1    Monti, G.2    Brenot, F.3
  • 9
    • 0037331116 scopus 로고    scopus 로고
    • Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis
    • Kawut S.M., Taichman D.B., Archer-Chicko C.L., et al. Hemodynamics and survival in patients with pulmonary arterial hypertension related to systemic sclerosis. Chest 2003, 123:344-350.
    • (2003) Chest , vol.123 , pp. 344-350
    • Kawut, S.M.1    Taichman, D.B.2    Archer-Chicko, C.L.3
  • 10
    • 33749329543 scopus 로고    scopus 로고
    • Clinical differences between idiopathic and scleroderma-related pulmonary hypertension
    • Fisher M.R., Mathai S.C., Champion H.C., et al. Clinical differences between idiopathic and scleroderma-related pulmonary hypertension. Arthritis Rheum 2006, 54:3043-3050.
    • (2006) Arthritis Rheum , vol.54 , pp. 3043-3050
    • Fisher, M.R.1    Mathai, S.C.2    Champion, H.C.3
  • 11
    • 58449122578 scopus 로고    scopus 로고
    • Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era
    • Condliffe R., Kiely D.G., Peacock A.J., et al. Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 2009, 179:151-157.
    • (2009) Am J Respir Crit Care Med , vol.179 , pp. 151-157
    • Condliffe, R.1    Kiely, D.G.2    Peacock, A.J.3
  • 12
    • 0346727461 scopus 로고    scopus 로고
    • Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis
    • Jimenez S.A., Derk C.T. Following the molecular pathways toward an understanding of the pathogenesis of systemic sclerosis. Ann Intern Med 2004, 140:37-50.
    • (2004) Ann Intern Med , vol.140 , pp. 37-50
    • Jimenez, S.A.1    Derk, C.T.2
  • 13
    • 0037785169 scopus 로고    scopus 로고
    • Systemic sclerosis: the susceptible host (genetics and environment)
    • Tan F.K. Systemic sclerosis: the susceptible host (genetics and environment). Rheum Dis Clin North Am 2003, 29:211-237.
    • (2003) Rheum Dis Clin North Am , vol.29 , pp. 211-237
    • Tan, F.K.1
  • 14
    • 0023854176 scopus 로고
    • Scleroderma (systemic sclerosis): classification, subsets and pathogenesis
    • LeRoy E.C., Black C., Fleischmajer R., et al. Scleroderma (systemic sclerosis): classification, subsets and pathogenesis. J Rheumatol 1988, 15:202-205.
    • (1988) J Rheumatol , vol.15 , pp. 202-205
    • LeRoy, E.C.1    Black, C.2    Fleischmajer, R.3
  • 15
    • 0038799783 scopus 로고    scopus 로고
    • Scleroderma epidemiology
    • Mayes M.D. Scleroderma epidemiology. Rheum Dis Clin North Am 2003, 29:239-254.
    • (2003) Rheum Dis Clin North Am , vol.29 , pp. 239-254
    • Mayes, M.D.1
  • 16
    • 0023784057 scopus 로고
    • An epidemiological study of scleroderma in the West Midlands
    • Silman A., Jannini S., Symmons D., et al. An epidemiological study of scleroderma in the West Midlands. Br J Rheumatol 1988, 27:286-290.
    • (1988) Br J Rheumatol , vol.27 , pp. 286-290
    • Silman, A.1    Jannini, S.2    Symmons, D.3
  • 17
    • 0025936232 scopus 로고
    • Epidemiological study of patients with systemic sclerosis in Tokyo
    • Tamaki T., Mori S., Takehara K. Epidemiological study of patients with systemic sclerosis in Tokyo. Arch Dermatol Res 1991, 283:366-371.
    • (1991) Arch Dermatol Res , vol.283 , pp. 366-371
    • Tamaki, T.1    Mori, S.2    Takehara, K.3
  • 18
    • 2442589580 scopus 로고    scopus 로고
    • A study of the prevalence of systemic sclerosis in northeast England
    • Allcock R.J., Forrest I., Corris P.A., et al. A study of the prevalence of systemic sclerosis in northeast England. Rheumatology (Oxford) 2004, 43:596-602.
    • (2004) Rheumatology (Oxford) , vol.43 , pp. 596-602
    • Allcock, R.J.1    Forrest, I.2    Corris, P.A.3
  • 19
    • 0043074631 scopus 로고    scopus 로고
    • Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population
    • Mayes M.D., Lacey J.V., Beebe-Dimmer J., et al. Prevalence, incidence, survival, and disease characteristics of systemic sclerosis in a large US population. Arthritis Rheum 2003, 48:2246-2255.
    • (2003) Arthritis Rheum , vol.48 , pp. 2246-2255
    • Mayes, M.D.1    Lacey, J.V.2    Beebe-Dimmer, J.3
  • 20
    • 29144536869 scopus 로고    scopus 로고
    • Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study
    • Hachulla E., Gressin V., Guillevin L., et al. Early detection of pulmonary arterial hypertension in systemic sclerosis: a French nationwide prospective multicenter study. Arthritis Rheum 2005, 52:3792-3800.
    • (2005) Arthritis Rheum , vol.52 , pp. 3792-3800
    • Hachulla, E.1    Gressin, V.2    Guillevin, L.3
  • 21
    • 0142187600 scopus 로고    scopus 로고
    • Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach
    • Mukerjee D., St George D., Coleiro B., et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis 2003, 62:1088-1093.
    • (2003) Ann Rheum Dis , vol.62 , pp. 1088-1093
    • Mukerjee, D.1    St George, D.2    Coleiro, B.3
  • 22
    • 0030463427 scopus 로고    scopus 로고
    • Prevalence of pulmonary hypertension in limited and diffuse scleroderma
    • Battle R.W., Davitt M.A., Cooper S.M., et al. Prevalence of pulmonary hypertension in limited and diffuse scleroderma. Chest 1996, 110:1515-1519.
    • (1996) Chest , vol.110 , pp. 1515-1519
    • Battle, R.W.1    Davitt, M.A.2    Cooper, S.M.3
  • 23
    • 0022600374 scopus 로고
    • Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis
    • Stupi A.M., Steen V.D., Owens G.R., et al. Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis. Arthritis Rheum 1986, 29:515-524.
    • (1986) Arthritis Rheum , vol.29 , pp. 515-524
    • Stupi, A.M.1    Steen, V.D.2    Owens, G.R.3
  • 24
    • 0029920461 scopus 로고    scopus 로고
    • Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3RNP antibody
    • Sacks D.G., Okano Y., Steen V.D., et al. Isolated pulmonary hypertension in systemic sclerosis with diffuse cutaneous involvement: association with serum anti-U3RNP antibody. J Rheumatol 1996, 23:639-642.
    • (1996) J Rheumatol , vol.23 , pp. 639-642
    • Sacks, D.G.1    Okano, Y.2    Steen, V.D.3
  • 25
    • 0035038492 scopus 로고    scopus 로고
    • Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival
    • MacGregor A.J., Canavan R., Knight C., et al. Pulmonary hypertension in systemic sclerosis: risk factors for progression and consequences for survival. Rheumatology (Oxford) 2001, 40:453-459.
    • (2001) Rheumatology (Oxford) , vol.40 , pp. 453-459
    • MacGregor, A.J.1    Canavan, R.2    Knight, C.3
  • 26
    • 65249161113 scopus 로고    scopus 로고
    • Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension
    • Fisher M.R., Forfia P.R., Chamera E., et al. Accuracy of Doppler echocardiography in the hemodynamic assessment of pulmonary hypertension. Am J Respir Crit Care Med 2009, 179:615-621.
    • (2009) Am J Respir Crit Care Med , vol.179 , pp. 615-621
    • Fisher, M.R.1    Forfia, P.R.2    Chamera, E.3
  • 27
    • 33646269035 scopus 로고    scopus 로고
    • Pulmonary arterial hypertension in France: results from a national registry
    • Humbert M., Sitbon O., Chaouat A., et al. Pulmonary arterial hypertension in France: results from a national registry. Am J Respir Crit Care Med 2006, 173:1023-1030.
    • (2006) Am J Respir Crit Care Med , vol.173 , pp. 1023-1030
    • Humbert, M.1    Sitbon, O.2    Chaouat, A.3
  • 28
    • 34447546600 scopus 로고    scopus 로고
    • An epidemiological study of pulmonary arterial hypertension
    • Peacock A.J., Murphy N.F., McMurray J.J., et al. An epidemiological study of pulmonary arterial hypertension. Eur Respir J 2007, 30:104-109.
    • (2007) Eur Respir J , vol.30 , pp. 104-109
    • Peacock, A.J.1    Murphy, N.F.2    McMurray, J.J.3
  • 29
    • 76749135710 scopus 로고    scopus 로고
    • Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry
    • Badesch D.B., Raskob G.E., Elliott C.G., et al. Pulmonary arterial hypertension: baseline characteristics from the REVEAL Registry. Chest 2010, 137:376-387.
    • (2010) Chest , vol.137 , pp. 376-387
    • Badesch, D.B.1    Raskob, G.E.2    Elliott, C.G.3
  • 30
    • 0029905937 scopus 로고    scopus 로고
    • Systemic sclerosis. A vascular perspective
    • LeRoy E.C. Systemic sclerosis. A vascular perspective. Rheum Dis Clin North Am 1996, 22:675-694.
    • (1996) Rheum Dis Clin North Am , vol.22 , pp. 675-694
    • LeRoy, E.C.1
  • 32
    • 0033758304 scopus 로고    scopus 로고
    • Endothelial cell apoptosis in systemic sclerosis is induced by antibody-dependent cell-mediated cytotoxicity via CD95
    • Sgonc R., Gruschwitz M.S., Boeck G., et al. Endothelial cell apoptosis in systemic sclerosis is induced by antibody-dependent cell-mediated cytotoxicity via CD95. Arthritis Rheum 2000, 43:2550-2562.
    • (2000) Arthritis Rheum , vol.43 , pp. 2550-2562
    • Sgonc, R.1    Gruschwitz, M.S.2    Boeck, G.3
  • 33
    • 0344511824 scopus 로고    scopus 로고
    • Blood coagulation, fibrinolysis, and markers of endothelial dysfunction in systemic sclerosis
    • Cerinic M.M., Valentini G., Sorano G.G., et al. Blood coagulation, fibrinolysis, and markers of endothelial dysfunction in systemic sclerosis. Semin Arthritis Rheum 2003, 32:285-295.
    • (2003) Semin Arthritis Rheum , vol.32 , pp. 285-295
    • Cerinic, M.M.1    Valentini, G.2    Sorano, G.G.3
  • 34
    • 0025808660 scopus 로고
    • Predictors of survival in systemic sclerosis (scleroderma)
    • Altman R.D., Medsger T.A., Bloch D.A., et al. Predictors of survival in systemic sclerosis (scleroderma). Arthritis Rheum 1991, 34:403-413.
    • (1991) Arthritis Rheum , vol.34 , pp. 403-413
    • Altman, R.D.1    Medsger, T.A.2    Bloch, D.A.3
  • 35
    • 0029614879 scopus 로고
    • Serial circulating adhesion molecule levels reflect disease severity in systemic sclerosis
    • Denton C.P., Bickerstaff M.C., Shiwen X., et al. Serial circulating adhesion molecule levels reflect disease severity in systemic sclerosis. Br J Rheumatol 1995, 34:1048-1054.
    • (1995) Br J Rheumatol , vol.34 , pp. 1048-1054
    • Denton, C.P.1    Bickerstaff, M.C.2    Shiwen, X.3
  • 36
    • 34247564964 scopus 로고    scopus 로고
    • Angiogenic and angiostatic factors in systemic sclerosis: increased levels of vascular endothelial growth factor are a feature of the earliest disease stages and are associated with the absence of fingertip ulcers
    • Distler O., Del Rosso A., Giacomelli R., et al. Angiogenic and angiostatic factors in systemic sclerosis: increased levels of vascular endothelial growth factor are a feature of the earliest disease stages and are associated with the absence of fingertip ulcers. Arthritis Res 2002, 4:R11.
    • (2002) Arthritis Res , vol.4
    • Distler, O.1    Del Rosso, A.2    Giacomelli, R.3
  • 37
    • 0038166874 scopus 로고    scopus 로고
    • Elevated vascular endothelial growth factor in systemic sclerosis
    • Choi J.J., Min D.J., Cho M.L., et al. Elevated vascular endothelial growth factor in systemic sclerosis. J Rheumatol 2003, 30:1529-1533.
    • (2003) J Rheumatol , vol.30 , pp. 1529-1533
    • Choi, J.J.1    Min, D.J.2    Cho, M.L.3
  • 38
    • 0033850755 scopus 로고    scopus 로고
    • Increased concentrations of the circulating angiogenesis inhibitor endostatin in patients with systemic sclerosis
    • Hebbar M., Peyrat J.P., Hornez L., et al. Increased concentrations of the circulating angiogenesis inhibitor endostatin in patients with systemic sclerosis. Arthritis Rheum 2000, 43:889-893.
    • (2000) Arthritis Rheum , vol.43 , pp. 889-893
    • Hebbar, M.1    Peyrat, J.P.2    Hornez, L.3
  • 39
    • 0026537043 scopus 로고
    • Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis
    • Okano Y., Steen V.D., Medsger T.A. Autoantibody to U3 nucleolar ribonucleoprotein (fibrillarin) in patients with systemic sclerosis. Arthritis Rheum 1992, 35:95-100.
    • (1992) Arthritis Rheum , vol.35 , pp. 95-100
    • Okano, Y.1    Steen, V.D.2    Medsger, T.A.3
  • 40
    • 0031951993 scopus 로고    scopus 로고
    • Antiendothelial cell antibodies in scleroderma correlate with severe digital ischemia and pulmonary arterial hypertension
    • Negi V.S., Tripathy N.K., Misra R., et al. Antiendothelial cell antibodies in scleroderma correlate with severe digital ischemia and pulmonary arterial hypertension. J Rheumatol 1998, 25:462-466.
    • (1998) J Rheumatol , vol.25 , pp. 462-466
    • Negi, V.S.1    Tripathy, N.K.2    Misra, R.3
  • 41
    • 0029118026 scopus 로고
    • Antibodies to fibrin bound tissue type plasminogen activator in systemic sclerosis
    • Fritzler M.J., Hart D.A., Wilson D., et al. Antibodies to fibrin bound tissue type plasminogen activator in systemic sclerosis. J Rheumatol 1995, 22:1688-1693.
    • (1995) J Rheumatol , vol.22 , pp. 1688-1693
    • Fritzler, M.J.1    Hart, D.A.2    Wilson, D.3
  • 42
    • 0031018254 scopus 로고    scopus 로고
    • Primary pulmonary hypertension, tissue plasminogen activator antibodies, and HLA-DQ7
    • Morse J.H., Barst R.J., Fotino M., et al. Primary pulmonary hypertension, tissue plasminogen activator antibodies, and HLA-DQ7. Am J Respir Crit Care Med 1997, 155:274-278.
    • (1997) Am J Respir Crit Care Med , vol.155 , pp. 274-278
    • Morse, J.H.1    Barst, R.J.2    Fotino, M.3
  • 43
    • 0033850582 scopus 로고    scopus 로고
    • Anti-topoisomerase II alpha autoantibodies in systemic sclerosis-association with pulmonary hypertension and HLA-B35
    • Grigolo B., Mazzetti I., Meliconi R., et al. Anti-topoisomerase II alpha autoantibodies in systemic sclerosis-association with pulmonary hypertension and HLA-B35. Clin Exp Immunol 2000, 121:539-543.
    • (2000) Clin Exp Immunol , vol.121 , pp. 539-543
    • Grigolo, B.1    Mazzetti, I.2    Meliconi, R.3
  • 44
    • 29244486514 scopus 로고    scopus 로고
    • Autoimmunity and pulmonary hypertension: a perspective
    • Nicolls M.R., Taraseviciene-Stewart L., Rai P.R., et al. Autoimmunity and pulmonary hypertension: a perspective. Eur Respir J 2005, 26:1110-1118.
    • (2005) Eur Respir J , vol.26 , pp. 1110-1118
    • Nicolls, M.R.1    Taraseviciene-Stewart, L.2    Rai, P.R.3
  • 45
    • 24744455197 scopus 로고    scopus 로고
    • Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension
    • Tamby M.C., Chanseaud Y., Humbert M., et al. Anti-endothelial cell antibodies in idiopathic and systemic sclerosis associated pulmonary arterial hypertension. Thorax 2005, 60:765-772.
    • (2005) Thorax , vol.60 , pp. 765-772
    • Tamby, M.C.1    Chanseaud, Y.2    Humbert, M.3
  • 46
    • 0036269002 scopus 로고    scopus 로고
    • Autoantibodies to fibroblasts induce a proadhesive and proinflammatory fibroblast phenotype in patients with systemic sclerosis
    • Chizzolini C., Raschi E., Rezzonico R., et al. Autoantibodies to fibroblasts induce a proadhesive and proinflammatory fibroblast phenotype in patients with systemic sclerosis. Arthritis Rheum 2002, 46:1602-1613.
    • (2002) Arthritis Rheum , vol.46 , pp. 1602-1613
    • Chizzolini, C.1    Raschi, E.2    Rezzonico, R.3
  • 47
    • 33845510782 scopus 로고    scopus 로고
    • Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension
    • Tamby M.C., Humbert M., Guilpain P., et al. Antibodies to fibroblasts in idiopathic and scleroderma-associated pulmonary hypertension. Eur Respir J 2006, 28:799-807.
    • (2006) Eur Respir J , vol.28 , pp. 799-807
    • Tamby, M.C.1    Humbert, M.2    Guilpain, P.3
  • 48
    • 43849113603 scopus 로고    scopus 로고
    • Identification of target antigens of antifibroblast antibodies in pulmonary arterial hypertension
    • Terrier B., Tamby M.C., Camoin L., et al. Identification of target antigens of antifibroblast antibodies in pulmonary arterial hypertension. Am J Respir Crit Care Med 2008, 177:1128-1134.
    • (2008) Am J Respir Crit Care Med , vol.177 , pp. 1128-1134
    • Terrier, B.1    Tamby, M.C.2    Camoin, L.3
  • 49
    • 33746379082 scopus 로고    scopus 로고
    • Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension
    • Sanchez O., Sitbon O., Jais X., et al. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 2006, 130:182-189.
    • (2006) Chest , vol.130 , pp. 182-189
    • Sanchez, O.1    Sitbon, O.2    Jais, X.3
  • 50
    • 0033838125 scopus 로고    scopus 로고
    • Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene
    • Deng Z., Morse J.H., Slager S.L., et al. Familial primary pulmonary hypertension (gene PPH1) is caused by mutations in the bone morphogenetic protein receptor-II gene. Am J Hum Genet 2000, 67:737-744.
    • (2000) Am J Hum Genet , vol.67 , pp. 737-744
    • Deng, Z.1    Morse, J.H.2    Slager, S.L.3
  • 51
    • 0033817459 scopus 로고    scopus 로고
    • Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium
    • Lane K.B., Machado R.D., Pauciulo M.W., et al. Heterozygous germline mutations in BMPR2, encoding a TGF-beta receptor, cause familial primary pulmonary hypertension. The International PPH Consortium. Nat Genet 2000, 26:81-84.
    • (2000) Nat Genet , vol.26 , pp. 81-84
    • Lane, K.B.1    Machado, R.D.2    Pauciulo, M.W.3
  • 52
    • 2942581061 scopus 로고    scopus 로고
    • Genetic basis of pulmonary arterial hypertension: current understanding and future directions
    • Newman J.H., Trembath R.C., Morse J.A., et al. Genetic basis of pulmonary arterial hypertension: current understanding and future directions. J Am Coll Cardiol 2004, 43:33S-39S.
    • (2004) J Am Coll Cardiol , vol.43
    • Newman, J.H.1    Trembath, R.C.2    Morse, J.A.3
  • 53
    • 24644475032 scopus 로고    scopus 로고
    • Low frequency of BMPR2 mutations in a German cohort of patients with sporadic idiopathic pulmonary arterial hypertension
    • Koehler R., Grunig E., Pauciulo M.W., et al. Low frequency of BMPR2 mutations in a German cohort of patients with sporadic idiopathic pulmonary arterial hypertension. J Med Genet 2004, 41:e127.
    • (2004) J Med Genet , vol.41
    • Koehler, R.1    Grunig, E.2    Pauciulo, M.W.3
  • 54
    • 0035683060 scopus 로고    scopus 로고
    • Genetic aspects of pulmonary arterial hypertension
    • Morse J.H., Deng Z., Knowles J.A. Genetic aspects of pulmonary arterial hypertension. Ann Med 2001, 33:596-603.
    • (2001) Ann Med , vol.33 , pp. 596-603
    • Morse, J.H.1    Deng, Z.2    Knowles, J.A.3
  • 55
    • 0035797556 scopus 로고    scopus 로고
    • Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia
    • Trembath R.C., Thomson J.R., Machado R.D., et al. Clinical and molecular genetic features of pulmonary hypertension in patients with hereditary hemorrhagic telangiectasia. N Engl J Med 2001, 345:325-334.
    • (2001) N Engl J Med , vol.345 , pp. 325-334
    • Trembath, R.C.1    Thomson, J.R.2    Machado, R.D.3
  • 56
    • 0036844311 scopus 로고    scopus 로고
    • Pulmonary hypertension in scleroderma spectrum of disease: lack of bone morphogenetic protein receptor 2 mutations
    • Morse J., Barst R., Horn E., et al. Pulmonary hypertension in scleroderma spectrum of disease: lack of bone morphogenetic protein receptor 2 mutations. J Rheumatol 2002, 29:2379-2381.
    • (2002) J Rheumatol , vol.29 , pp. 2379-2381
    • Morse, J.1    Barst, R.2    Horn, E.3
  • 57
    • 0036822548 scopus 로고    scopus 로고
    • Mutations of bone morphogenetic protein receptor type II are not found in patients with pulmonary hypertension and underlying connective tissue diseases
    • Tew M.B., Arnett F.C., Reveille J.D., et al. Mutations of bone morphogenetic protein receptor type II are not found in patients with pulmonary hypertension and underlying connective tissue diseases. Arthritis Rheum 2002, 46:2829-2830.
    • (2002) Arthritis Rheum , vol.46 , pp. 2829-2830
    • Tew, M.B.1    Arnett, F.C.2    Reveille, J.D.3
  • 58
    • 11944251003 scopus 로고    scopus 로고
    • The -2518 promoter polymorphism in the MCP-1 gene is associated with systemic sclerosis
    • Karrer S., Bosserhoff A.K., Weiderer P., et al. The -2518 promoter polymorphism in the MCP-1 gene is associated with systemic sclerosis. J Invest Dermatol 2005, 124(1):92-98.
    • (2005) J Invest Dermatol , vol.124 , Issue.1 , pp. 92-98
    • Karrer, S.1    Bosserhoff, A.K.2    Weiderer, P.3
  • 59
    • 10444261953 scopus 로고    scopus 로고
    • Association of a functional CD19 polymorphism with susceptibility to systemic sclerosis
    • Tsuchiya N., Kuroki K., Fujimoto M., et al. Association of a functional CD19 polymorphism with susceptibility to systemic sclerosis. Arthritis Rheum 2004, 50:4002-4007.
    • (2004) Arthritis Rheum , vol.50 , pp. 4002-4007
    • Tsuchiya, N.1    Kuroki, K.2    Fujimoto, M.3
  • 60
    • 10044289478 scopus 로고    scopus 로고
    • -238 and +489 TNF-alpha along with TNF-RII gene polymorphisms associate with the diffuse phenotype in patients with systemic sclerosis
    • Tolusso B., Fabris M., Caporali R., et al. -238 and +489 TNF-alpha along with TNF-RII gene polymorphisms associate with the diffuse phenotype in patients with systemic sclerosis. Immunol Lett 2005, 96:103-108.
    • (2005) Immunol Lett , vol.96 , pp. 103-108
    • Tolusso, B.1    Fabris, M.2    Caporali, R.3
  • 61
    • 0037231514 scopus 로고    scopus 로고
    • Association of IL1A gene polymorphisms with susceptibility to and severity of systemic sclerosis in the Japanese population
    • Kawaguchi Y., Tochimoto A., Ichikawa N., et al. Association of IL1A gene polymorphisms with susceptibility to and severity of systemic sclerosis in the Japanese population. Arthritis Rheum 2003, 48:186-192.
    • (2003) Arthritis Rheum , vol.48 , pp. 186-192
    • Kawaguchi, Y.1    Tochimoto, A.2    Ichikawa, N.3
  • 62
    • 0347949601 scopus 로고    scopus 로고
    • Interleukin 1alpha single-nucleotide polymorphism associated with systemic sclerosis
    • Hutyrova B., Lukac J., Bosak V., et al. Interleukin 1alpha single-nucleotide polymorphism associated with systemic sclerosis. J Rheumatol 2004, 31:81-84.
    • (2004) J Rheumatol , vol.31 , pp. 81-84
    • Hutyrova, B.1    Lukac, J.2    Bosak, V.3
  • 63
    • 0242440942 scopus 로고    scopus 로고
    • Analysis of the 5' flanking region of the interleukin 10 gene in patients with systemic sclerosis
    • Crilly A., Hamilton J., Clark C.J., et al. Analysis of the 5' flanking region of the interleukin 10 gene in patients with systemic sclerosis. Rheumatology (Oxford) 2003, 42:1295-1298.
    • (2003) Rheumatology (Oxford) , vol.42 , pp. 1295-1298
    • Crilly, A.1    Hamilton, J.2    Clark, C.J.3
  • 64
    • 0141676373 scopus 로고    scopus 로고
    • Genome-wide association study for regions of systemic sclerosis susceptibility in a Choctaw Indian population with high disease prevalence
    • Zhou X., Tan F.K., Wang N., et al. Genome-wide association study for regions of systemic sclerosis susceptibility in a Choctaw Indian population with high disease prevalence. Arthritis Rheum 2003, 48:2585-2592.
    • (2003) Arthritis Rheum , vol.48 , pp. 2585-2592
    • Zhou, X.1    Tan, F.K.2    Wang, N.3
  • 65
    • 34047123228 scopus 로고    scopus 로고
    • Association between an endoglin gene polymorphism and systemic sclerosis-related pulmonary arterial hypertension
    • Wipff J., Kahan A., Hachulla E., et al. Association between an endoglin gene polymorphism and systemic sclerosis-related pulmonary arterial hypertension. Rheumatology (Oxford) 2007, 46:622-625.
    • (2007) Rheumatology (Oxford) , vol.46 , pp. 622-625
    • Wipff, J.1    Kahan, A.2    Hachulla, E.3
  • 66
    • 0347624457 scopus 로고    scopus 로고
    • Age and risk of pulmonary arterial hypertension in scleroderma
    • Schachna L., Wigley F.M., Chang B., et al. Age and risk of pulmonary arterial hypertension in scleroderma. Chest 2003, 124:2098-2104.
    • (2003) Chest , vol.124 , pp. 2098-2104
    • Schachna, L.1    Wigley, F.M.2    Chang, B.3
  • 67
    • 32144461709 scopus 로고    scopus 로고
    • Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma
    • Chang B., Schachna L., White B., et al. Natural history of mild-moderate pulmonary hypertension and the risk factors for severe pulmonary hypertension in scleroderma. J Rheumatol 2006, 33:269-274.
    • (2006) J Rheumatol , vol.33 , pp. 269-274
    • Chang, B.1    Schachna, L.2    White, B.3
  • 68
    • 0037331671 scopus 로고    scopus 로고
    • Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement
    • Steen V., Medsger T.A. Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement. Arthritis Rheum 2003, 48:516-522.
    • (2003) Arthritis Rheum , vol.48 , pp. 516-522
    • Steen, V.1    Medsger, T.A.2
  • 69
    • 38149040974 scopus 로고    scopus 로고
    • High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis
    • Allanore Y., Borderie D., Avouac J., et al. High N-terminal pro-brain natriuretic peptide levels and low diffusing capacity for carbon monoxide as independent predictors of the occurrence of precapillary pulmonary arterial hypertension in patients with systemic sclerosis. Arthritis Rheum 2008, 58:284-291.
    • (2008) Arthritis Rheum , vol.58 , pp. 284-291
    • Allanore, Y.1    Borderie, D.2    Avouac, J.3
  • 70
    • 73649099833 scopus 로고    scopus 로고
    • Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension
    • Shah A.A., Wigley F.M., Hummers L.K. Telangiectases in scleroderma: a potential clinical marker of pulmonary arterial hypertension. J Rheumatol 2010, 37(1):98-104.
    • (2010) J Rheumatol , vol.37 , Issue.1 , pp. 98-104
    • Shah, A.A.1    Wigley, F.M.2    Hummers, L.K.3
  • 71
    • 33646113362 scopus 로고    scopus 로고
    • Right heart function in scleroderma: insights from myocardial Doppler tissue imaging
    • Hsiao S.H., Lee C.Y., Chang S.M., et al. Right heart function in scleroderma: insights from myocardial Doppler tissue imaging. J Am Soc Echocardiogr 2006, 19:507-514.
    • (2006) J Am Soc Echocardiogr , vol.19 , pp. 507-514
    • Hsiao, S.H.1    Lee, C.Y.2    Chang, S.M.3
  • 72
    • 33846837374 scopus 로고    scopus 로고
    • Right heart function and scleroderma: insights from tricuspid annular plane systolic excursion
    • Lee C.Y., Chang S.M., Hsiao S.H., et al. Right heart function and scleroderma: insights from tricuspid annular plane systolic excursion. Echocardiography 2007, 24:118-125.
    • (2007) Echocardiography , vol.24 , pp. 118-125
    • Lee, C.Y.1    Chang, S.M.2    Hsiao, S.H.3
  • 73
    • 45349108255 scopus 로고    scopus 로고
    • Cardiac involvement in systemic sclerosis assessed by tissue-Doppler echocardiography during routine care: a controlled study of 100 consecutive patients
    • Meune C., Avouac J., Wahbi K., et al. Cardiac involvement in systemic sclerosis assessed by tissue-Doppler echocardiography during routine care: a controlled study of 100 consecutive patients. Arthritis Rheum 2008, 58:1803-1809.
    • (2008) Arthritis Rheum , vol.58 , pp. 1803-1809
    • Meune, C.1    Avouac, J.2    Wahbi, K.3
  • 74
    • 49649095907 scopus 로고    scopus 로고
    • Right ventricular contractility in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension
    • Overbeek M.J., Lankhaar J.W., Westerhof N., et al. Right ventricular contractility in systemic sclerosis-associated and idiopathic pulmonary arterial hypertension. Eur Respir J 2008, 31:1160-1166.
    • (2008) Eur Respir J , vol.31 , pp. 1160-1166
    • Overbeek, M.J.1    Lankhaar, J.W.2    Westerhof, N.3
  • 75
    • 33745725935 scopus 로고    scopus 로고
    • Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension
    • Williams M.H., Handler C.E., Akram R., et al. Role of N-terminal brain natriuretic peptide (N-TproBNP) in scleroderma-associated pulmonary arterial hypertension. Eur Heart J 2006, 27:1485-1494.
    • (2006) Eur Heart J , vol.27 , pp. 1485-1494
    • Williams, M.H.1    Handler, C.E.2    Akram, R.3
  • 76
    • 44949228252 scopus 로고    scopus 로고
    • Hyponatremia predicts right heart failure and poor survival in pulmonary arterial hypertension
    • Forfia P.R., Mathai S.C., Fisher M.R., et al. Hyponatremia predicts right heart failure and poor survival in pulmonary arterial hypertension. Am J Respir Crit Care Med 2008, 177:1364-1369.
    • (2008) Am J Respir Crit Care Med , vol.177 , pp. 1364-1369
    • Forfia, P.R.1    Mathai, S.C.2    Fisher, M.R.3
  • 77
    • 33745637694 scopus 로고    scopus 로고
    • Systemic sclerosis associated pulmonary hypertension: improved survival in the current era
    • Williams M.H., Das C., Handler C.E., et al. Systemic sclerosis associated pulmonary hypertension: improved survival in the current era. Heart 2006, 92:926-932.
    • (2006) Heart , vol.92 , pp. 926-932
    • Williams, M.H.1    Das, C.2    Handler, C.E.3
  • 78
    • 0029985117 scopus 로고    scopus 로고
    • Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients
    • Koh E.T., Lee P., Gladman D.D., et al. Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients. Br J Rheumatol 1996, 35:989-993.
    • (1996) Br J Rheumatol , vol.35 , pp. 989-993
    • Koh, E.T.1    Lee, P.2    Gladman, D.D.3
  • 79
    • 0037126044 scopus 로고    scopus 로고
    • Survival in primary pulmonary hypertension: the impact of epoprostenol therapy
    • McLaughlin V.V., Shillington A., Rich S. Survival in primary pulmonary hypertension: the impact of epoprostenol therapy. Circulation 2002, 106:1477-1482.
    • (2002) Circulation , vol.106 , pp. 1477-1482
    • McLaughlin, V.V.1    Shillington, A.2    Rich, S.3
  • 80
    • 34347266509 scopus 로고    scopus 로고
    • Changes in causes of death in systemic sclerosis, 1972-2002
    • Steen V.D., Medsger T.A. Changes in causes of death in systemic sclerosis, 1972-2002. Ann Rheum Dis 2007, 66:940-944.
    • (2007) Ann Rheum Dis , vol.66 , pp. 940-944
    • Steen, V.D.1    Medsger, T.A.2
  • 81
    • 43249099112 scopus 로고    scopus 로고
    • An update in pulmonary hypertension in systemic lupus erythematosus-do we need to know about it?
    • Pope J. An update in pulmonary hypertension in systemic lupus erythematosus-do we need to know about it?. Lupus 2008, 17:274-277.
    • (2008) Lupus , vol.17 , pp. 274-277
    • Pope, J.1
  • 82
    • 11444249795 scopus 로고    scopus 로고
    • Pulmonary hypertension associated with systemic lupus erythematosus
    • [discussion: 997], [in French]
    • Haas C. Bull Acad Natl Med 2004, 188:985-997. [discussion: 997], [in French].
    • (2004) Bull Acad Natl Med , vol.188 , pp. 985-997
    • Haas, C.1
  • 83
    • 0021331405 scopus 로고
    • A prospective evaluation emphasizing pulmonary involvement in patients with mixed connective tissue disease
    • Sullivan W.D., Hurst D.J., Harmon C.E., et al. A prospective evaluation emphasizing pulmonary involvement in patients with mixed connective tissue disease. Medicine (Baltimore) 1984, 63:92-107.
    • (1984) Medicine (Baltimore) , vol.63 , pp. 92-107
    • Sullivan, W.D.1    Hurst, D.J.2    Harmon, C.E.3
  • 84
    • 34548678641 scopus 로고    scopus 로고
    • Pulmonary arterial hypertension: a rare complication of primary Sjögren syndrome: report of 9 new cases and review of the literature
    • Launay D., Hachulla E., Hatron P.Y., et al. Pulmonary arterial hypertension: a rare complication of primary Sjögren syndrome: report of 9 new cases and review of the literature. Medicine (Baltimore) 2007, 86:299-315.
    • (2007) Medicine (Baltimore) , vol.86 , pp. 299-315
    • Launay, D.1    Hachulla, E.2    Hatron, P.Y.3
  • 85
    • 0029055964 scopus 로고
    • Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension
    • Giaid A., Saleh D. Reduced expression of endothelial nitric oxide synthase in the lungs of patients with pulmonary hypertension. N Engl J Med 1995, 333:214-221.
    • (1995) N Engl J Med , vol.333 , pp. 214-221
    • Giaid, A.1    Saleh, D.2
  • 86
    • 0033032949 scopus 로고    scopus 로고
    • Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension
    • Tuder R.M., Cool C.D., Geraci M.W., et al. Prostacyclin synthase expression is decreased in lungs from patients with severe pulmonary hypertension. Am J Respir Crit Care Med 1999, 159:1925-1932.
    • (1999) Am J Respir Crit Care Med , vol.159 , pp. 1925-1932
    • Tuder, R.M.1    Cool, C.D.2    Geraci, M.W.3
  • 87
    • 0027324745 scopus 로고
    • Expression of endothelin-1 in the lungs of patients with pulmonary hypertension
    • Giaid A., Yanagisawa M., Langleben D., et al. Expression of endothelin-1 in the lungs of patients with pulmonary hypertension. N Engl J Med 1993, 328:1732-1739.
    • (1993) N Engl J Med , vol.328 , pp. 1732-1739
    • Giaid, A.1    Yanagisawa, M.2    Langleben, D.3
  • 88
    • 0026632785 scopus 로고
    • The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension
    • Rich S., Kaufmann E., Levy P.S. The effect of high doses of calcium-channel blockers on survival in primary pulmonary hypertension. N Engl J Med 1992, 327:76-81.
    • (1992) N Engl J Med , vol.327 , pp. 76-81
    • Rich, S.1    Kaufmann, E.2    Levy, P.S.3
  • 89
    • 20544448927 scopus 로고    scopus 로고
    • Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension
    • Sitbon O., Humbert M., Jais X., et al. Long-term response to calcium channel blockers in idiopathic pulmonary arterial hypertension. Circulation 2005, 111:3105-3111.
    • (2005) Circulation , vol.111 , pp. 3105-3111
    • Sitbon, O.1    Humbert, M.2    Jais, X.3
  • 90
    • 66549127501 scopus 로고    scopus 로고
    • 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
    • McLaughlin V.V., Archer S.L., Badesch D.B., 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. Circulation 2009, 119(16):2250-2294.
    • (2009) Circulation , vol.119 , Issue.16 , pp. 2250-2294
    • McLaughlin, V.V.1    Archer, S.L.2    Badesch, D.B.3
  • 91
    • 0025334367 scopus 로고
    • Regulatory functions of the vascular endothelium
    • Vane J.R., Anggard E.E., Botting R.M. Regulatory functions of the vascular endothelium. N Engl J Med 1990, 323:27-36.
    • (1990) N Engl J Med , vol.323 , pp. 27-36
    • Vane, J.R.1    Anggard, E.E.2    Botting, R.M.3
  • 92
    • 0030031888 scopus 로고    scopus 로고
    • A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group
    • Barst R.J., Rubin L.J., Long W.A., et al. A comparison of continuous intravenous epoprostenol (prostacyclin) with conventional therapy for primary pulmonary hypertension. The Primary Pulmonary Hypertension Study Group. N Engl J Med 1996, 334:296-302.
    • (1996) N Engl J Med , vol.334 , pp. 296-302
    • Barst, R.J.1    Rubin, L.J.2    Long, W.A.3
  • 93
    • 0032576672 scopus 로고    scopus 로고
    • Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension
    • McLaughlin V.V., Genthner D.E., Panella M.M., et al. Reduction in pulmonary vascular resistance with long-term epoprostenol (prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 1998, 338:273-277.
    • (1998) N Engl J Med , vol.338 , pp. 273-277
    • McLaughlin, V.V.1    Genthner, D.E.2    Panella, M.M.3
  • 94
    • 0025356811 scopus 로고
    • Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial
    • Rubin L.J., Mendoza J., Hood M., et al. Treatment of primary pulmonary hypertension with continuous intravenous prostacyclin (epoprostenol). Results of a randomized trial. Ann Intern Med 1990, 112:485-491.
    • (1990) Ann Intern Med , vol.112 , pp. 485-491
    • Rubin, L.J.1    Mendoza, J.2    Hood, M.3
  • 95
    • 17144452827 scopus 로고    scopus 로고
    • Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial
    • Badesch D.B., Tapson V.F., McGoon M.D., et al. Continuous intravenous epoprostenol for pulmonary hypertension due to the scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med 2000, 132:425-434.
    • (2000) Ann Intern Med , vol.132 , pp. 425-434
    • Badesch, D.B.1    Tapson, V.F.2    McGoon, M.D.3
  • 96
    • 70450162190 scopus 로고    scopus 로고
    • Longterm survival among patients with scleroderma-associated pulmonary arterial hypertension treated with intravenous epoprostenol
    • Badesch D.B., McGoon M.D., Barst R.J., et al. Longterm survival among patients with scleroderma-associated pulmonary arterial hypertension treated with intravenous epoprostenol. J Rheumatol 2009, 36:2244-2249.
    • (2009) J Rheumatol , vol.36 , pp. 2244-2249
    • Badesch, D.B.1    McGoon, M.D.2    Barst, R.J.3
  • 97
    • 0037086133 scopus 로고    scopus 로고
    • Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial
    • Simonneau G., Barst R.J., Galie N., et al. Continuous subcutaneous infusion of treprostinil, a prostacyclin analogue, in patients with pulmonary arterial hypertension: a double-blind, randomized, placebo-controlled trial. Am J Respir Crit Care Med 2002, 165:800-804.
    • (2002) Am J Respir Crit Care Med , vol.165 , pp. 800-804
    • Simonneau, G.1    Barst, R.J.2    Galie, N.3
  • 98
    • 33645119109 scopus 로고    scopus 로고
    • Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial
    • Tapson V.F., Gomberg-Maitland M., McLaughlin V.V., et al. Safety and efficacy of IV treprostinil for pulmonary arterial hypertension: a prospective, multicenter, open-label, 12-week trial. Chest 2006, 129:683-688.
    • (2006) Chest , vol.129 , pp. 683-688
    • Tapson, V.F.1    Gomberg-Maitland, M.2    McLaughlin, V.V.3
  • 99
    • 0032939165 scopus 로고    scopus 로고
    • Pulmonary edema during acute infusion of epoprostenol in a patient with pulmonary hypertension and limited scleroderma
    • Farber H.W., Graven K.K., Kokolski G., et al. Pulmonary edema during acute infusion of epoprostenol in a patient with pulmonary hypertension and limited scleroderma. J Rheumatol 1999, 26:1195-1196.
    • (1999) J Rheumatol , vol.26 , pp. 1195-1196
    • Farber, H.W.1    Graven, K.K.2    Kokolski, G.3
  • 100
    • 0031983972 scopus 로고    scopus 로고
    • Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease
    • Palmer S.M., Robinson L.J., Wang A., et al. Massive pulmonary edema and death after prostacyclin infusion in a patient with pulmonary veno-occlusive disease. Chest 1998, 113:237-240.
    • (1998) Chest , vol.113 , pp. 237-240
    • Palmer, S.M.1    Robinson, L.J.2    Wang, A.3
  • 101
    • 0036875131 scopus 로고    scopus 로고
    • Emerging medical therapies for pulmonary arterial hypertension
    • Galie N., Manes A., Branzi A. Emerging medical therapies for pulmonary arterial hypertension. Prog Cardiovasc Dis 2002, 45:213-224.
    • (2002) Prog Cardiovasc Dis , vol.45 , pp. 213-224
    • Galie, N.1    Manes, A.2    Branzi, A.3
  • 102
    • 0035818316 scopus 로고    scopus 로고
    • Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study
    • Channick R.N., Simonneau G., Sitbon O., et al. Effects of the dual endothelin-receptor antagonist bosentan in patients with pulmonary hypertension: a randomised placebo-controlled study. Lancet 2001, 358:1119-1123.
    • (2001) Lancet , vol.358 , pp. 1119-1123
    • Channick, R.N.1    Simonneau, G.2    Sitbon, O.3
  • 103
    • 0037149718 scopus 로고    scopus 로고
    • Bosentan therapy for pulmonary arterial hypertension
    • Rubin L.J., Badesch D.B., Barst R.J., et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med 2002, 346:896-903.
    • (2002) N Engl J Med , vol.346 , pp. 896-903
    • Rubin, L.J.1    Badesch, D.B.2    Barst, R.J.3
  • 104
    • 33750344748 scopus 로고    scopus 로고
    • Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions
    • Denton C.P., Humbert M., Rubin L., et al. Bosentan treatment for pulmonary arterial hypertension related to connective tissue disease: a subgroup analysis of the pivotal clinical trials and their open-label extensions. Ann Rheum Dis 2006, 65:1336-1340.
    • (2006) Ann Rheum Dis , vol.65 , pp. 1336-1340
    • Denton, C.P.1    Humbert, M.2    Rubin, L.3
  • 105
    • 25844531004 scopus 로고    scopus 로고
    • Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases
    • Girgis R.E., Mathai S.C., Krishnan J.A., et al. Long-term outcome of bosentan treatment in idiopathic pulmonary arterial hypertension and pulmonary arterial hypertension associated with the scleroderma spectrum of diseases. J Heart Lung Transplant 2005, 24:1626-1631.
    • (2005) J Heart Lung Transplant , vol.24 , pp. 1626-1631
    • Girgis, R.E.1    Mathai, S.C.2    Krishnan, J.A.3
  • 106
    • 47949095665 scopus 로고    scopus 로고
    • Bosentan regulates the expression of adhesion molecules on circulating T cells and serum soluble adhesion molecules in systemic sclerosis-associated pulmonary arterial hypertension
    • Iannone F., Riccardi M.T., Guiducci S., et al. Bosentan regulates the expression of adhesion molecules on circulating T cells and serum soluble adhesion molecules in systemic sclerosis-associated pulmonary arterial hypertension. Ann Rheum Dis 2008, 67:1121-1126.
    • (2008) Ann Rheum Dis , vol.67 , pp. 1121-1126
    • Iannone, F.1    Riccardi, M.T.2    Guiducci, S.3
  • 107
    • 33746819503 scopus 로고    scopus 로고
    • Bosentan for the treatment of systemic sclerosis-associated pulmonary arterial hypertension, pulmonary fibrosis and digital ulcers
    • Jain M., Varga J. Bosentan for the treatment of systemic sclerosis-associated pulmonary arterial hypertension, pulmonary fibrosis and digital ulcers. Expert Opin Pharmacother 2006, 7:1487-1501.
    • (2006) Expert Opin Pharmacother , vol.7 , pp. 1487-1501
    • Jain, M.1    Varga, J.2
  • 108
    • 34948865560 scopus 로고    scopus 로고
    • Selective endothelin a receptor antagonism with sitaxsentan for pulmonary arterial hypertension associated with connective tissue disease
    • Girgis R.E., Frost A.E., Hill N.S., et al. Selective endothelin a receptor antagonism with sitaxsentan for pulmonary arterial hypertension associated with connective tissue disease. Ann. Rheum. Dis. 2007, 66(11):1467-1472.
    • (2007) Ann. Rheum. Dis. , vol.66 , Issue.11 , pp. 1467-1472
    • Girgis, R.E.1    Frost, A.E.2    Hill, N.S.3
  • 109
    • 46449124591 scopus 로고    scopus 로고
    • Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2
    • Galie N., Olschewski H., Oudiz R.J., et al. Ambrisentan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controlled, multicenter, efficacy (ARIES) study 1 and 2. Circulation 2008, 117:3010-3019.
    • (2008) Circulation , vol.117 , pp. 3010-3019
    • Galie, N.1    Olschewski, H.2    Oudiz, R.J.3
  • 110
    • 26444534291 scopus 로고    scopus 로고
    • Sildenafil citrate therapy for pulmonary arterial hypertension
    • Galie N., Ghofrani H.A., Torbicki A., et al. Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 2005, 353:2148-2157.
    • (2005) N Engl J Med , vol.353 , pp. 2148-2157
    • Galie, N.1    Ghofrani, H.A.2    Torbicki, A.3
  • 111
    • 36849073290 scopus 로고    scopus 로고
    • Sildenafil for pulmonary arterial hypertension associated with connective tissue disease
    • Badesch D.B., Hill N.S., Burgess G., et al. Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol 2007, 34:2417-2422.
    • (2007) J Rheumatol , vol.34 , pp. 2417-2422
    • Badesch, D.B.1    Hill, N.S.2    Burgess, G.3
  • 112
    • 67649523052 scopus 로고    scopus 로고
    • Tadalafil therapy for pulmonary arterial hypertension
    • Galie N., Brundage B.H., Ghofrani H.A., et al. Tadalafil therapy for pulmonary arterial hypertension. Circulation 2009, 119:2894-2903.
    • (2009) Circulation , vol.119 , pp. 2894-2903
    • Galie, N.1    Brundage, B.H.2    Ghofrani, H.A.3
  • 113
    • 33845526777 scopus 로고    scopus 로고
    • Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension
    • McLaughlin V.V., Oudiz R.J., Frost A., et al. Randomized study of adding inhaled iloprost to existing bosentan in pulmonary arterial hypertension. Am J Respir Crit Care Med 2006, 174:1257-1263.
    • (2006) Am J Respir Crit Care Med , vol.174 , pp. 1257-1263
    • McLaughlin, V.V.1    Oudiz, R.J.2    Frost, A.3
  • 114
    • 10444246635 scopus 로고    scopus 로고
    • Combination therapy with bosentan and sildenafil in idiopathic pulmonary arterial hypertension
    • Hoeper M.M., Faulenbach C., Golpon H., et al. Combination therapy with bosentan and sildenafil in idiopathic pulmonary arterial hypertension. Eur Respir J 2004, 24:1007-1010.
    • (2004) Eur Respir J , vol.24 , pp. 1007-1010
    • Hoeper, M.M.1    Faulenbach, C.2    Golpon, H.3
  • 115
    • 54549125950 scopus 로고    scopus 로고
    • Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial
    • Simonneau G., Rubin L.J., Galie N., et al. Addition of sildenafil to long-term intravenous epoprostenol therapy in patients with pulmonary arterial hypertension: a randomized trial. Ann Intern Med 2008, 149:521-530.
    • (2008) Ann Intern Med , vol.149 , pp. 521-530
    • Simonneau, G.1    Rubin, L.J.2    Galie, N.3
  • 116
    • 33847743922 scopus 로고    scopus 로고
    • Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension
    • Mathai S.C., Girgis R.E., Fisher M.R., et al. Addition of sildenafil to bosentan monotherapy in pulmonary arterial hypertension. Eur Respir J 2007, 29:469-475.
    • (2007) Eur Respir J , vol.29 , pp. 469-475
    • Mathai, S.C.1    Girgis, R.E.2    Fisher, M.R.3
  • 117
    • 0021193273 scopus 로고
    • Primary pulmonary hypertension: natural history and the importance of thrombosis
    • Fuster V., Steele P.M., Edwards W.D., et al. Primary pulmonary hypertension: natural history and the importance of thrombosis. Circulation 1984, 70:580-587.
    • (1984) Circulation , vol.70 , pp. 580-587
    • Fuster, V.1    Steele, P.M.2    Edwards, W.D.3
  • 118
    • 20444501324 scopus 로고    scopus 로고
    • Lessons learned from cancer may help in the treatment of pulmonary hypertension
    • Adnot S. Lessons learned from cancer may help in the treatment of pulmonary hypertension. J Clin Invest 2005, 115:1461-1463.
    • (2005) J Clin Invest , vol.115 , pp. 1461-1463
    • Adnot, S.1
  • 119
    • 26444540768 scopus 로고    scopus 로고
    • Reversal of experimental pulmonary hypertension by PDGF inhibition
    • Schermuly R.T., Dony E., Ghofrani H.A., et al. Reversal of experimental pulmonary hypertension by PDGF inhibition. J Clin Invest 2005, 115:2811-2821.
    • (2005) J Clin Invest , vol.115 , pp. 2811-2821
    • Schermuly, R.T.1    Dony, E.2    Ghofrani, H.A.3
  • 120
    • 40749109217 scopus 로고    scopus 로고
    • Genomic assessment of a multikinase inhibitor, sorafenib, in a rodent model of pulmonary hypertension
    • Moreno-Vinasco L., Gomberg-Maitland M., Maitland M.L., et al. Genomic assessment of a multikinase inhibitor, sorafenib, in a rodent model of pulmonary hypertension. Physiol Genomics 2008, 33:278-291.
    • (2008) Physiol Genomics , vol.33 , pp. 278-291
    • Moreno-Vinasco, L.1    Gomberg-Maitland, M.2    Maitland, M.L.3
  • 121
    • 77956709644 scopus 로고    scopus 로고
    • Imatinib in pulmonary arterial hypertension patients with inadequate response to established therapy
    • Ghofrani H.A., Morrell N.W., Hoeper M.M., et al. Imatinib in pulmonary arterial hypertension patients with inadequate response to established therapy. Am J Respir Crit Care Med 2010, 182(9):1171-1177.
    • (2010) Am J Respir Crit Care Med , vol.182 , Issue.9 , pp. 1171-1177
    • Ghofrani, H.A.1    Morrell, N.W.2    Hoeper, M.M.3
  • 122
    • 40849083461 scopus 로고    scopus 로고
    • Identification of candidate genes in scleroderma-related pulmonary arterial hypertension
    • Grigoryev D.N., Mathai S.C., Fisher M.R., et al. Identification of candidate genes in scleroderma-related pulmonary arterial hypertension. Transl Res 2008, 151:197-207.
    • (2008) Transl Res , vol.151 , pp. 197-207
    • Grigoryev, D.N.1    Mathai, S.C.2    Fisher, M.R.3
  • 123
    • 63449091795 scopus 로고    scopus 로고
    • Significant improvement of right ventricular function by imatinib mesylate in scleroderma-associated pulmonary arterial hypertension
    • ten Freyhaus H., Dumitrescu D., Bovenschulte H., et al. Significant improvement of right ventricular function by imatinib mesylate in scleroderma-associated pulmonary arterial hypertension. Clin Res Cardiol 2009, 98:265-267.
    • (2009) Clin Res Cardiol , vol.98 , pp. 265-267
    • ten Freyhaus, H.1    Dumitrescu, D.2    Bovenschulte, H.3
  • 124
    • 33845660778 scopus 로고    scopus 로고
    • Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension
    • Schachna L., Medsger T.A., Dauber J.H., et al. Lung transplantation in scleroderma compared with idiopathic pulmonary fibrosis and idiopathic pulmonary arterial hypertension. Arthritis Rheum 2006, 54:3954-3961.
    • (2006) Arthritis Rheum , vol.54 , pp. 3954-3961
    • Schachna, L.1    Medsger, T.A.2    Dauber, J.H.3
  • 125
    • 77957848190 scopus 로고    scopus 로고
    • Systemic sclerosis and bilateral lung transplantation: a single center experience
    • Saggar R., Khanna D., Furst D.E., et al. Systemic sclerosis and bilateral lung transplantation: a single center experience. Eur Respir J 2010, 36(4):893-900.
    • (2010) Eur Respir J , vol.36 , Issue.4 , pp. 893-900
    • Saggar, R.1    Khanna, D.2    Furst, D.E.3
  • 126
    • 34248389766 scopus 로고    scopus 로고
    • Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases
    • Dorfmuller P., Humbert M., Perros F., et al. Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases. Hum Pathol 2007, 38:893-902.
    • (2007) Hum Pathol , vol.38 , pp. 893-902
    • Dorfmuller, P.1    Humbert, M.2    Perros, F.3


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