Intensive Immunosuppressive Therapy Improves Pulmonary Hemodynamics and Long-Term Prognosis in Patients With Pulmonary Arterial Hypertension Associated With Connective Tissue Disease
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概要
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Background: Pulmonary arterial hypertension (PAH) remains a serious disease characterized by elevated pulmonary artery pressure (PAP) and increased pulmonary vascular resistance (PVR). Among its subtypes, PAH associated with connective tissue disease (CPAH) has the worse prognosis, because of resistance to conventional vasodilator therapy. We hypothesized that intensive immunosuppressive therapy (IIT) could improve the pulmonary hemodynamics in CPAH. Methods and Results: In our pulmonary hypertension (PH) cohort of 182 patients, we evaluated 13 consecutive patients with CPAH who received IIT combined with cyclophosphamide and glucocorticosteroids (IIT group, mean age 45±8 years, 12 females and 1 male). We compared them with 8 historical controls (control group: mean age 52±18 years, 8 females) for pulmonary hemodynamics and prognosis. Both groups were treated with conventional vasodilator therapy. Although the mean PAP (mPAP) remained unchanged in the control group, IIT significantly decreased mPAP (40±9 to 29±11mmHg, P<0.01) and tended to decrease PVR (700±434 to 481±418 dyne·s·cm-5, P=0.07). Importantly, in 6 of the 13 patients in the IIT group, mPAP was almost normalized (<25mmHg) and remained stabilized for more than 1 year. Furthermore, the IIT group showed significantly better prognosis compared with the control group (P<0.01). Conclusions: These results suggest that IIT as well as conventional vasodilator therapy improves the pulmonary hemodynamics and long-term prognosis of patients with CPAH. (Circ J 2011; 75: 2668-2674)
- 2011-10-25
著者
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Fukumoto Yoshihiro
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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下川 宏明
東北大学大学院医学系研究科循環器病態学
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下川 宏明
東北大学循環器内科学
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下川 宏明
九州大学 院工
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下川 宏明
東北大学医学系研究科循環器病態学
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Sakamoto Kazuhiko
Division Of Internal Medicine Saito Hospital
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SHIRATO Kunio
Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
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Shimada Kazuyuki
山口大学 医学系研究科器官病態内科学
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Shimada Kazuyuki
大阪府立病院機構大阪府立急性期総合医療センター
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Shirato Kunio
山口大学 医学系研究科器官病態内科学
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Shimamoto Kazuaki
札幌医科大学 医学部内科学第二
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Shimamoto Kazuaki
()
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Shimokawa Hiroaki
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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Shirato K
Department Of Internal Medicine Ii Sapporo Medical University
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Kawano Satoru
Medicine Of Cardiovascular Pathophysiology Graduate School Of Comprehensive Human Science University
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TAWARA Shunsuke
Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
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Sugimura Koichiro
Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
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Miura Yutaka
Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
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TATEBE Shunsuke
Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
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Aoki Tatsuo
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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Sugimoto Ken
東北大学 臨床薬学
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Sugimoto Ken
Department Of Geriatric Medicine Osaka University Graduate School Of Medicine
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Shimoda Kazuya
Division Of Cardiovascular Medicine Jichi Medical University School Of Medicine
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Shirato Kunio
Department Of Internal Medicine Ii Sapporo Medical University
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Sugimura Kohichiro
東北大学 医学系研究科循環器病態学分野
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Sugimura Koichiro
東北大学 循環器内科
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Ishii Tomonori
Department Of Hematology And Rheumatology Tohoku University Graduate School Of Medicine
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Shimokawa Hiroaki
Juntendo Sports Clinic Juntendo University Hospital
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SHIMAMOTO Kazuaki
Committee for Epidemiology and Clinical Management of Atherosclerosis
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Shirato Kunio
東北大学 第1内科
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下川 宏明
東北大学 大学院医学系研究科循環器病態学分野
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Shimamoto Kazuaki
帝京大学 医学部内科
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Shimokawa Hiroaki
Research Institute Of Angiocardiology And Cardiovascular Clinic Faculty Of Medicine Kyushu Universit
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Shioji Keisuke
国立循環器病センター 疫学部
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Shioji Keisuke
Department Of Epidemiology National Cardiovascular Center
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Shimada K
Division Of Cardiovascular Medicine Department Of Medicine Jichi Medical University
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Simada Kazuyuki
南郷村国民健康保険病院
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下川 弘晃
東海農政局
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Sugimura Kohichiro
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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Shimada Kazuyuki
Division Of Cardiovascular Medicine Jichi Medical School
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Kawano Satoru
Division Of Cardiology Institute Of Clinical Medicine University Of Tsukuba
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Shimokawa Hiroaki
Research Institute Of Angiocardiology And Cardiovascular Clinic Kyushu University School Of Medicine
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Miura Yutaka
Department Of Agricultural Chemistry Faculty Of Agriculture The University Of Tokyo
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Shimamoto Kazuaki
Second Department Of Internal Medicine Sapporo Medical College
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Shimokawa Hiroaki
Department Of Cardiovascular Medicine Kyushu University Graduate School Of Medicine
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Takagi Atsutoshi
Department Of Cardiovascular Medicine Juntendo University School Of Medicine
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Shimamoto Kazuaki
2nd Department Of Internal Medicine Sapporo Medical University
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Shimamoto Kazuaki
The 2nd Department Of Internal Medicine Sapporo Medical University School Of Medicine
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Sugimura Kouichiro
Department Of Cardiovascular Medicine Tohoku University Graduated School Of Medicine
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Shimokawa Hiroaki
Department Of Cardiology Tohoku University School Of Medicine
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Shimokawa Hiroaki
Kyushu University Graduate School Of Medical Sciences
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Satoh Kiichi
Department Of Internal Medicine Division Of Gastroenterology Jichi Medical University
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Shimamoto Kazuaki
Second Department Of Internal Medicine (cardiovascular Medicine) Sapporo Medical University School O
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Sugano K
Department Of Internal Medicine Division Of Gastroenterology Jichi Medical University
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Nochioka Kotaro
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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Kawano Satoru
Cardiovascular Division Institute Of Clinical Medicine Graduate School Of Comprehensive Human Scienc
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Shimamoto Kazuaki
The Seceond Department Of Internal Medicine Sapporo Medical University School Of Medicine
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Shimada Kazuyuki
The Departments Of Cardiology Echi Medical School
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Satoh Kimio
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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Simada Kazuyuki
Division Of Cardiovascular Medicine Department Of Medicine Jichi Medical School
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Shimokawa Hiroaki
Department Of Cardiovascular Medicine Kyushu University Graduate School Of Medical Science
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Fukumoto Yoshihiro
Department Of Cardiovascular Medicine Kyushu University Graduate School Of Medical Sciences
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Suzuka Hiroshi
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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Shimokawa Hiroaki
The Department Of Cardiovascular Medicine Kyushu University Graduate School Of Medical Science
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Shimamoto Kazuaki
The Second Department Of Internal Medicine Sapporo Medical College
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Kawano Satoru
Cardiovascular Division Institute Of Clinical Medicine Graduate School Of Comprehensive Human Scienc
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Shimokawa Hiroaki
From Reseach Institute Of Angiocardiology And Cardiovascular Clinic Faculty Of Medicine Kyushu Unive
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Shimokawa Hiroaki
Department Of Cardiovascular Medicine Graduate School Of Medical Sciences Kyushu University
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Shimokawa Hiroaki
The Research Institute Of Angiocardiology And Cardiovascular Clinic Kyushu University School Of Medi
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Shimokawa Hiroaki
The Research Institute Of Angiocardiology And Cardiovascular Clinic Kyusyu Univarsity School Of Medi
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Shimamoto Kazuyuki
Second Department Of Internal Medicine Sapporo Medical University School Of Medicine
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Do. E
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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MIYAMICHI YAMAMOTO
Department of Cardiovascular Medicine, Tohoku University Graduate School of Medicine
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Sato Kouichi
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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Miyamichi Yamamoto
Department Of Cardiovascular Medicine Tohoku University Graduate School Of Medicine
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下川 宏明
東北大学大学院 医学系研究科 循環器内科学
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Kawano Satoru
Medicine Of Cardiovascular Pathophysiology Graduate School Of Comprehensive Human Science University Of Tsukuba
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Fukumoto Yoshihiro
Department of Cardiovascular Medicine and Department of Evidence-based Cardiovascular Medicine, Tohoku University Graduate School of Medicine
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Nochioka Kotaro
Department of Cardiovascular Medicine and Department of Evidence-based Cardiovascular Medicine, Tohoku University Graduate School of Medicine
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