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1)Shirai Y, Yasuoka, Okano Y, et al. Clinical characteristics and survival of Japanese patients with connective tissue disease and pulmonary arterial hypertension: a single-centre cohort. Rheumatology. 2012; 51: 1846-54
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2)Launay D, Hachulla E, Hatron PY, et al. Pulmonary arterial hypertension: a rare complication of primary Sjo¨gren syndrome: report of 9 new cases and review of the literature. Medicine. 2007; 86: 299-315
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3)Jais X, Launay D, Yaici A, et al. Immunosuppressive therapy in lupus- and mixed connective tissue disease-associated pulmonary arterial hypertension: a retrospective analysis of twenty-three cases. Arthritis Rheum. 2008; 58: 521-31
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4)Chung L, Farber HW, Benza R, et al. Unique predictors of mortality in patients with pulmonary arterial hypertension associated with systemic sclerosis in the REVEAL Registry. Chest. 2014; 146: 1494-504
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5)Halliday SJ, Hemnes AR, Robbins IM, et al. Prognostic value of acute vasodilator response in pulmonary arterial hypertension: beyond the "classic" responders. J Heart Lung Transplant. 2015; 34: 312-8
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6)Hinchcliff M, Fischer A, Schiopu E, et al. Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): baseline characteristics and description of study population. J Rheumatol. 2011; 38: 2172-9
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7)Fox BD, Shimony A, Langleben D, et al. High prevalence of occult left heart disease in scleroderma-pulmonary hypertension. Eur Respir J. 2013; 42: 1083-91
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8)Sanchez O, Sitbon O, Jaïs X, et al. Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest. 2006; 130: 182-9
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9)Galiè N, Barbera JA, Frost A, et al. AMBITION: a randomised, multicentre study of first-line ambrisentan and tadalafil combination therapy in subjects with pulmonary arterial hypertension (PAH). Eur Respir J. 2014; 44(Suppl 58): A2916
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10)Coghlan JG, Galiè N, Barbera JA, et al. Initial combination therapy of ambrisentan and tadalafil in connective tissue disease associated pulmonary arterial hypertension (CTD-PAH): subgroup analysis from the AMBITION trial. Ann Rheum Dis. 2015; 74(Suppl 2): 173
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11)National Institutes of Health Clinical Center. A randomized, double-blind, placebo-controlled, phase II multicenter trial of a monoclonal antibody to CD20 (rituximab) for the treatment of systemic sclerosis-associated pulmonary arterial hypertension (SSc-PAH). NCT01086540. Bethesda, MD: National Institutes of Health; 2012. http://clinicaltrials.gov/ct2/show/record/NCT01086540. Accessed December 12, 2012
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12)Takeshima Y, Iwasaki Y. Kato R, et al. Immunosuppressive therapy is promising for pulmonary arterial hypertension associated with connective tissue disease (CTD-PAH). Ann Rheum Dis. 2015; 74(Suppl 2): 1079
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