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2) Barst RJ, Gibbs JSR, Ghofrani HA, et al. Updated evidence-based treatment algorithm in pulmonary arterial hypertension. J Am Coll Cardiol. 2009; 54: S78-84
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3) Akagi A, Nakamura K, Miyaji K, et al. Marked hemodynamic improvements by high-dose epoprostenol therapy in patients with idiopathic pulmonary arterial hypertension. Circ J. 2010; 74: 2200-5
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4) Ghofrani HA, Hoeper MM, Halank M, et al. Riociguat for chronic thromboembolic pulmonary hypertension and pulmonary arterial hypertension: a phase II study. Eur Respir J. 2010; 36: 792-9
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5) Ghofrani HA, Morrell NW, Hoeper MM, et al. Imatinib in pulmonary arterial hypertension patients with inadequate response to established therapy. Am J Respir Crit Care Med. 2010; 182: 1171-7
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6) Muketjee D, St George D, Coliro B, et al. Prevalence and outcome in systemic sclerosis associated pulmonary arterial hypertension: application of a registry approach. Ann Rheum Dis. 2003; 62: 1088-93
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7) 吉田俊治, 深谷修作. 膠原病性肺高血圧症の頻度と病態の解析. 厚生労働科学研究費補助金免疫アレルギー疾患予防・治療研究事業全身性自己免疫疾患における難治性病態の診断と治療法に関する研究. 平成15年度総括・分担研究報告書. 2004; 40-43
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8) Jais X, Launay D, Yaici A, et al. Immunosuppressive therapy in lupus and mixedconnective tissue disease associated pulmonary arterialhypertension-A retrospective analysis of twenty-three cases–. Arthritis Rheum. 2008; 58: 521-31
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9) Chung L, Liu J, Parsons L, et al. Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL. Chest. 2010; 138: 1383-94
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10) Campo A, Mathai SC, Pavec JL, et al. Hemodynamic predictors of survival in scleroderma-related pulmonary arterial hypertension. Am J Respir Crit Care Med. 2010; 182; 252-60
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11) Mathai SC, Hummers LK, Champion HC, et al. Survival in pulmonary hypertension associated with the scleroderma spectrum of diseases–Impact of interstitial lung disease–. Arthritis Rheum. 2009; 60: 569-77
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12) Impens AJ, Wangkaew S, Seibold JR. The 6-minute walk test in scleroderma-how measuring everything measures nothing. Rheumatology (Oxford). 2008; 47(suppl 5): v68
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13) Rubin LJ, Badesch DB, Barst RJ, et al. Bosentan therapy for pulmonary arterial hypertension. N Engl J Med. 2002; 346: 896-903
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14) Galie N, Olschewski H, Oudiz RJ, et al. Ambrisen-tan for the treatment of pulmonary arterial hypertension: results of the ambrisentan in pulmonary arterial hypertension, randomized, double-blind, placebo-controled, multicenter, efficacy (ARIES) study 1 and 2. Circulation. 2008; 117: 3010-9
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15) Girgis RE, Frost AE, Hills NS, et al. Selective endothelin A receptor antagonism with sitaxsentan for pulmonary arterial hypertension associated with connective tissue disease. Ann Rheum Dis. 2007; 66: 1467-72
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16) Denton CP, Pope JE, Peter HH, et al. Long-term effects of bosentan on quality of life, survival, safety and tolerability in pulmonary arterial hypertension related connective tissue disease. An Rheum Dis. 2008; 67: 1222-8
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18) Badesch DB, Hills NS, Burgess G, et al. Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol. 2007; 34: 2417-22
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19) Badesch DB, Tapson VF, McGoon MD, et al. Continuous intravenous epoprostenol for pulmonary hypertension due to scleroderma spectrum of disease. A randomized, controlled trial. Ann Intern Med. 2000; 132: 425-34
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20) Badesch DB, McGoon MD, Barst RJ, et al. Long term survival among patients with scleroderma-associated pulmonary arterial hypertension treated with intravenous epoprostenol. J Rheumatol. 2009; 36: 2244-9
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24) 坂巻文雄, 京谷晋吾, 中西宣文. 換気障害または低酸素血症に伴う肺高血圧症. 日本呼吸器学会雑誌. 2009; 47(増): 20
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25) Le Pavec J, Girgis RE, Lechtzin N, et al. Systemic sclerosis related pulmonary hypertension associated with interstitial lung disease: Impact of pulmonary arterial hypertension therapies. Arthritis Rheum. 2011 May 2 [Epub ahead of print]
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26) Ebina M, Shimizukawa M, Shibata N, et al. Heterogeneous increase in CD34-positive alveolar capillaries in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2004; 169: 1203-8
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27) Farkas L, Gauldie J, Voelkel NF, et al. Pulmonary hypertension and idiopathic pulmonary fibrosis–A tale of angiogenesis, apoptosis and growth factors. Am J Respir Cell Mol Biol. 2010 Nov 5. [Epub ahead of print]
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28) King TE Jr, Behr J, Brown KK, et al. BUILD-1: a randomized placebo-controlled trial of bosentan in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2008; 177: 75-81
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29) The Idiopathic Pulmonary Fibrosis Clinical Research Network. A controlled trial of sildenafil in advanced idiopathic pulmonary fibrosis. N Engl J Med. 2010; 363: 620-8
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30) Pavec JL, Souza R, Herve P, et al. Portopulmonary hypertension –survival and prognostic factors–. Am J Respir Crit Care Med. 2008; 178: 637-43
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31) Sitbon O, Lascoux-Combe C, Delfraissy JF, et al. Prevalence of HIV-related pulmonary arterial hypertension in the current antiretroviral therapy era. Am J Respir Crit Care Med. 2008; 177: 108-13
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32) Long-term effects of bosentan in patients with HIV-associated pulmonary arterial hypertension. Euro Respir J. 2009; 33: 92-8
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