肺高血圧症Q&A

出版社: 先端医学社
著者:
発行日: 2015-10-01
分野: 臨床医学:内科  >  呼吸器一般
ISBN: 9784865501186
電子書籍版: 2015-10-01 (第1版第1刷)
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かつて、肺高血圧症は有効な治療法がなく、きわめて予後不良な疾患であった。しかし、病態の解明とともに治療薬の開発が進み、予後改善が期待されるようになった。一方、治療の遅れは生命予後に数年以上の差を生み出すこともわかり、その早期診断・治療の重要性はますます高まっている。本書は肺高血圧症を最初に診察する実地医家の先生方に、できるだけ早期に診断し、治療することの重要性を理解していただきたいと考え、企画されたものである。肺高血圧症の病態・診断・治療について、本症の専門家が「Q&A」形式でわかりやすく解説している。すべての実地医家の診療机に置いておきたい一冊。

目次

  • 肺高血圧症Q&A

    ―目次―

    Part 1 肺高血圧症治療の現状と疫学的背景をみる
     Q1. 肺高血圧症とはどのような疾患なのでしょうか。
        最新の定義について教えてください。
     Q2. 肺高血圧症にはどのような分類があるのでしょうか。
        また、それぞれどのくらいの頻度と予後でしょうか。

    Part 2 肺高血圧症の早期診断をめざす
     Q3. 肺高血圧症の早期診断はなぜ必要なのでしょうか。
        治療介入のタイミングが予後に及ぼす影響について
        教えてください。
     Q4. 肺高血圧症の診断はどのようにおこなうのでしょうか。
        診断アルゴリズム、専門医紹介のタイミングについて
        教えてください。
     Q5. 肺高血圧症の早期診断のためにはどのような問診や身体所見
        に着目すべきでしょうか。
        肺高血圧症を疑うべきポイントについて教えてください。
     …など9項目

    Part 3 肺高血圧症の病態をみる
     Q12. 特発性肺動脈性肺高血圧症(IPAH)および遺伝性肺動脈性
        肺高血圧症(HPAH)の病態、治療指針の概略と予後に
        ついて教えてください。
     Q13. 膠原病に伴う肺動脈性肺高血圧症の病態、
        治療指針と予後について教えてください。
     Q14. IgG4 関連疾患、キャッスルマン病、高安動脈炎に伴う肺高
        血圧症の病態、治療指針と予後について教えてください。
     …など11項目

    Part 4 肺高血圧症の治療戦略を探る
     Q23. 肺高血圧症と診断された場合、ニース分類や日本および
        世界のガイドラインを踏まえ、どのような治療アルゴ
        リズムが提唱されているのでしょうか。
     Q24. 肺高血圧症患者の日常生活指導のポイントと、
        QOLを低下させないための工夫を教えてください。
     Q25. 成人患者に対するプロスタサイクリン製剤の在宅持続静注
        療法の実際、管理のコツについて教えてください。
     …など22項目

この書籍の参考文献

参考文献のリンクは、リンク先の都合等により正しく表示されない場合がありますので、あらかじめご了承下さい。

本参考文献は電子書籍掲載内容を元にしております。

Part 1 肺高血圧症治療の現状と疫学的背景をみる

P.23 掲載の参考文献
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2) Rubin LJ, Mendoza J, Hood M et al:Treatment of primary pulmonary hypertension with continuous intrave-nous prostacyclin(epoprostenol). Results of a randomized trial. Ann Intern Med 112:485-491, 1990
3) McLaughlin VV, Genthner DE, Panella MM et al:Reduction in pulmonary vascular resistance with long-term epoprostenol(prostacyclin) therapy in primary pulmonary hypertension. N Engl J Med 338:273-277, 1998
4) Sasayama S, Kunieda T, Tomoike H et al:Effects of the endothelin receptor antagonist bosentan on hemody-namics, symptoms and functional capacity in Japanese patients with severe pulmonary hypertension. Circ J 69:131-137, 2005
5) Galie N, Ghofrani HA, Torbicki A et al:Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 353:2148-2157, 2005
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P.28 掲載の参考文献
1) Simonneau G, Gatzoulis MA, Adatia I et al:Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D34-D41, 2013
2) Galie N, Hoeper MM, Humbert M et al:Guidelines for the diagnosis and treatment of pulmonary hyperten-sion:the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology(ESC) and the European Respiratory Society(ERS), endorsed by the International Society of Heart and Lung Transplantation(ISHLT). Eur Heart J 30:2493-2537, 2009
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Part 2 肺高血圧症の早期診断をめざす

P.34 掲載の参考文献
1) D'Alonzo GE, Barst RJ, Ayres SM et al:Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 115:343-349, 1991
2) Benza RL, Miller DP, Barst RJ et al:An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest 142:448-456, 2012
3) Benza RL, Miller DP, Gomberg-Maitland M et al:Predicting survival in pulmonary arterial hypertension:insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Man-agement(REVEAL). Circulation 122:164-172, 2010
4) Rubin LJ, Badesch DB, Fleming TR et al:Long-term treatment with sildenafil citrate in pulmonary arterial hypertension:the SUPER-2 study. Chest 140:1274-1283, 2011
5) Galie N, Ghofrani HA, Torbicki A et al:Sildenafil citrate therapy for pulmonary arterial hypertension. N Engl J Med 353:2148-2157, 2005
6) Sanchez O, Sitbon O, Jais X et al:Immunosuppressive therapy in connective tissue diseases-associated pulmonary arterial hypertension. Chest 130:182-189, 2006
P.39 掲載の参考文献
1) Hoeper MM, Bogaard HJ, Condliffe R et al:Definitions and diagnosis of pulmonary hypertension.J Am Coll Cardiol 62(25 Suppl):D42-D50, 2013
P.44 掲載の参考文献
1) 国枝武義:「肺高血圧症の概念, 病態, 治療体制の確立に向けて」基調講演. Ther Res 26:2012-2021, 2005
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P.49 掲載の参考文献
1) Galie N, Hoeper MM, Humbert M et al:Guidelines for the diagnosis and treatment of pulmonary hypertension. Eur Respir J 34:1219-1263, 2009
2) 合同研究班(班長:中西宣文):循環器病の診断と診療に関するガイドライン(2011年度合同研究班報告)肺高血圧症治療ガイドライン(2012年改訂版), 2012
3) Foris V, Kovacs G, Tscherner M et al:Biomarkers in pulmonary hypertension:what do we know? Chest 144:274-283, 2013
4) Nagaya N, Nishikimi T, Uematsu M et al:Plasma brain natriuretic peptide as a prognostic indicator in patients with primary pulmonary hypertension. Circulation 102:865-870, 2000
5) Benza RL, Miller DP, Gomberg-Maitland M et al:Predicting survival in pulmonary arterial hypertension:insights from the Registry to Evaluate Early and Long-Term Pulmonary Arterial Hypertension Disease Man-agement(REVEAL). Circulation 122:164-172, 2010
6) Nagaya N, Uematsu M, Satoh T et al:Serum uric acid levels correlate with the severity and the mortality of primary pulmonary hypertension. Am J Respir Crit Care Med 160:487-492, 1999
7) Jinnin M, Ihn H, Yamane K et al:Plasma plasmin-alpha2-plasmin inhibitor complex levels are increased in systemic sclerosis patients with pulmonary hypertension. Rheumatology(Oxford) 42:240-243, 2003
8) Sakamaki F, Kyotani S, Nagaya N et al:Increase in thrombomodulin concentrations after pulmonary thrombo-endarterectomy in chronic thromboembolic pulmonary hypertension. Chest 124:1305-1311, 2003
9) Quarck R, Nawrot T, Meyns B et al:C-reactive protein:a new predictor of adverse outcome in pulmonary arterial hypertension. J Am Coll Cardiol 53:1211-1218, 2009
10) Hampole CV, Mehrotra AK, Thenappan T et al:Usefulness of red cell distribution width as a prognostic marker in pulmonary hypertension. Am J Cardiol 104:868-872, 2009
11) Cella G, Vianello F, Cozzi F et al:Effect of bosentan on plasma markers of endothelial cell activity in patients with secondary pulmorlary hypertension related to connective tissue diseases. J Rheumatol 36:760-767, 2009
12) Naito A, Tanabe N, Jujo T et al:Pentraxin3 in chronic thromboembolic pulmonary hypertension:a new bio-marker for screening from remitted pulmonary thromboembolism. PLoS One 9:e113086, 2014
13) Soon E, Holmes AM Treacy CM et al:Elevated levels of inflammatory cytokines predict survival in idiopathic and familial pulmonary arterial hypertension. Circulation l22:920-927, 2010
14) Selimovic N, Bergh CH, Andersson B et al:Growth factors and interleukin-6 across the lung circulation in pulmorlary hypertension. Eur Respir J 34:662-668, 2009
15) Lorenzen JM, Nickel N, Kramer R et al:Osteopontin in patients with idiopathic pulmonary hypertension. Chest 139:1010-1017, 2011
16) Nickel N, Jonigk D, Kempf T et al:GDF-15 is abundantly expressed in plexiform lesions in patients with pulmonary arterial hypertension and affects proliferation and apoptosis of pulmonary endothelial cells. Respir Res 12:62, 2011
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18) Rubens C, Ewert R, Halank M et al:Big endothelin-1 and endothelin-1 plasma levels are correlated with the severity of primary pulmonary hypertension. Chest 120:1562-1569, 2001
19) Kim J, Kang Y, Kojima Y et al:An endothelial apelin-FGF link mediated by miR-424 and miR-503 is disrupted in pulmonary arterial hypertension. Nat Med 19:74-82, 2013
20) Skoro-Sajer N, Mittermayer F, Panzenboeck A et al:Asymmetric dimethylarginine is increased in chronic thromboembolic pulmonary hypertension. Am J Respir Crit Care Med 176:1154-1160, 2007
21) Kumpers P, Nickel N, Lukasz A et al:Circulating angiopoietins in idiopathic pulmonary arterial hypertension. Eur Heart J 31:2291-2300, 2010
22) Bull TM, Golpon H, Hebbel RP et al:Circulating endothelial cells in pulmonary hypertension. Thromb Haemost 90:698-703, 2003
23) Smadja DM, Gaussem P, Mauge L et al:Circulating endothelial cells:a new candidate biomarker of irrevers-ible pulmonary hypertension secondary to congenital heart disease. Circulation 119:374-381, 2009
24) Toshner M, Voswinckel R, Southwood M et al:Evidence of dysfunction of endothelial progenitors in pulmonary arterial hypertension. Am J Respir Crit Care Med 180:780-787, 2009
25) Junhui Z, Xingxiang W, Guosheng F et al:Reduced number and activity of circulating endothelial progenitor cells in patients with idiopathic pulmonary arterial hypertension. Respir Med 102:1073-1079, 2008
P.54 掲載の参考文献
1) 合同研究班(班長:中西宣文):循環器病の診断と診療に関するガイドライン(2011年度合同研究班報告)肺高血圧症治療ガイドライン(2012年改訂版), 2012
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P.65 掲載の参考文献
1) McLaughlin VV, Archer SL, Badesch DB 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. J Am Coll Cardiol 53:1573-1619, 2009
2) Galie N, Hoeper MM, Humbert M et al:Guidelines for the diagnosis and treatment of pulmonary hyperten-sion:the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology(ESC) and the European Respiratory Society(ERS), endorsed by the International Society of Heart and Lung Transplantation(ISHLT). Eur Heart J 30:2493-2537, 2009
3) Humbert M, Sitbon O, Chaouat A et al:Pulmonary arterial hypertension in France:results from a national registry. Am J Respir Crit Care Med 173:1023-1030, 2006
4) Hachulla E, Gressin V, Guillevin L et al:Early detection of pulmonary arterial hypertension in systemic scle-rosis:a French nationwide prospective multicenter study. Arthritis Rheum 52:3792-3800, 2005
5) Condliffe R, Kiely DG, Peacock AJ et al:Connective tissue disease-associated pulmonary arterial hypertension in the modern treatment era. Am J Respir Crit Care Med 179:151-157, 2009
6) Naeije R, Vanderpool R Dhakal BP et al:Exercise-induced pulmonary hypertension:physiological basis and methodological concerns. Am J Respir Crit Care Med 187:576-583, 2013
7) Maron BA, Cockrill BA, Waxman AB et al:The invasive cardiopulmonary exercise test. Circulation 127:1157-1164, 2013
8) Rudski LG, Lai WW, Afilalo J et al:Guidelines for the echocardiographic assessment of the right heart in adults:a report from the American Society of Echocardiography endorsed by the European Association of Echocardiography, a registered branch of the European Society of Cardiology, and the Canadian Society of Echocardiography. J Am Soc Echocardiogr 23:685-713, 2010
9) Bossone E, D'Andrea A, D'Alto M et al:Echocardiography in pulmonary arterial hypertension:from diagnosis to prognosis. J Am Soc Echocardiogr 26:1-14, 2013
10) Janda S, Shahidi N, Gin K et al:Diagnostic accuracy of echocardiography for pulmonary hypertension:a sys-tematic review and meta-analysis. Heart 97:612-622, 2011
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12) Lam CS, Roger VL, Rodeheffer RJ et al:Pulmonary hypertension in heart failure with preserved ejection frac-tion:a community-based study. J Am Coll Cardiol 53:1119-1126, 2009
13) Lang RM, Badano LP, Mor-Avi V et al:Recommendations for cardiac chamber quantification by echocardiog-raphy in adults:an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr 28:1-39, 2015
P.69 掲載の参考文献
1) D'Alonzo GE, Barst RJ, Ayres SM et al:Survival in patients with primary pulmonary hypertension. Results from a national prospective registry. Ann Intern Med 115:343-349, 1991
2) Kovacs G, Avian A, Olschewski A et al:Zero reference level for right heart catheterisation. Eur. Respir J 42:1586-1594, 2013
3) Hoeper MM, Bogaard HJ, Condliffe R et al:Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D42-D50, 2013
4) Nakayama Y, Nakanishi N, Hayashi T et al:Pulmonary artery reflection for differentially diagnosing primary pulmonary hypertension and chronic pulmonary thromboembolism. J Am Coll Cardiol 38:214-218, 2001
P.74 掲載の参考文献
1) Hoeper MM, Bogaard HJ, Condliffe R et al:Definitions and diagnosis of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D42-D50, 2013
2) Reichelt A, Hoeper MM, Galanski M et al:Chronic thromboembolic pulmonary hypertension:evaluation with 64-detector row CT versus digital substraction angiography. Eur J Radiol 71:49-54, 2009
3) He J, Fang W, Lv B et al:Diagnosis of chronic thromboembolic pulmonary hypertension:comparison of ven-tilation/perfusion scanning and multidetector computed tomography pulmonary angiography with pulmonary angiography. Nucl Med Commun 33:459-463, 2012
4) Hoeper MM, Lee SH, Voswinckel R et al:Complications of right heart catheterization procedures in patients with pulmonary hypertension in experienced centers. J Am Coll Cardiol 48:2546-2552, 2006
5) Sugiyama M, Fukuda T, Sanda Y et al:Organized thrombus in pulmonary arteries in patients with chronic thromboembolic pulmonary hypertension;imaging with cone beam computed tomography. Jpn J Radiol 32:375-382, 2014
P.78 掲載の参考文献
1) Barst RJ, McGoon M, Torbicki A et al:Diagnosis and differential assessment of pulmonary arterial hypertension. J Am Coll Cardiol 43(12 SupPl S):40S-47S, 2004
2) 合同研究班(班長:中西宣文):循環器病の診断と診療に関するガイドライン(2011年度合同研究班報告)肺高血圧症治療ガイドライン(2012年改訂版), 2012
3) Galie N, Hoeper MM, Humbert M et al:Guidelines for the diagnosis and treatment of pulmonary hyperten-sion:the Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology(ESC) and the European Respiratory Society(ERS), endorsed by the International Society of Heart and Lung Transplantation(ISHLT). Eur Heart J 30:2493-2537, 2009
4) Sitbon O, Humbert M, Nunes H et al:Long-term intravenous epoprostenol infusion in primary pulmonary hypertension:prognostic factors and survival. J Am Coll Cardiol 40:780-788, 2002
5) Ogawa A, Ejiri K Matsubara H:Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan. Life Sci 118:414-419, 2014
6) Yamaki S, Kumate M, Yonesaka S et al:Lung biopsy diagnosis of operative indication in secundum atrial sep-tal defect with severe pulmonary vascular disease. Chest 126:1042-1047, 2004
7) Yamaki S:Pulmonary vascular disease associated with pulmonary hypertension in 445 patients:diagnosis from lung biopsy and autopsy. Gen Thorac Cardiovasc Surg 61:24-31, 2013
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Part 3 肺高血圧症の病態をみる

P.86 掲載の参考文献
1) 合同研究班(班長:中西宣文):循環器病の診断と診療に関するガイドライン(2011年度合同研究班報告)肺高血圧症治療ガイドライン(2012年改訂版), 2012
2) Ogawa A, Ejiri K, Matsubara H:Long-term patient survival with idiopathic/heritable pulmonary arterial hypertension treated at a single center in Japan. Life Sci 118:414-419, 2014
P.91 掲載の参考文献
1) Simonneau G, Gatzoulis MA, Adatia I et al:Updated clinical classification of pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl):D34-D41, 2013
2) Benza RL, Miller DP, Barst RJ et al:An evaluation of long-term survival from time of diagnosis in pulmonary arterial hypertension from the REVEAL Registry. Chest 142:448-456, 2012
3) Chung L, Liu J, Parsons L et al:Characterization of connective tissue disease-associated pulmonary arterial hypertension from REVEAL:identifying systemic sclerosis as a unique phenotype. Chest 138:1383-1394, 2010
4) Launay D, Sitbon O, Le Pavec J et al:Long-term outcome of systemic sclerosis-associated pulmonary arterial hypertension treated with bosentan as first-line monotherapy followed or not by the addition of prostanoids or sildenafil. Rheumatology(Oxford) 49:490-500, 2010
5) Condliffe R, Kiely DG, Peacock AJ et al:Connective tissue disease-associated pulmonary arteriai hypertension in the modern treatment era. Am J Respir Crit Care Med 179:151-157, 2009
6) Denton CP, 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 65:1336-1340, 2006
7) Badesch DB, Hill NS, Burgess G et al:Sildenafil for pulmonary arterial hypertension associated with connective tissue disease. J Rheumatol 34:2417-2422, 2007
8) Humbert M, Yaici A, de Groote P et al:Screening for pulmonary arterial hypertension in patients with systemic sclerosis:clinical characteristics at diagnosis and long-term survival. Arthritis Rheum 63:3522-3530, 2011
9) 川口鎮司:膠原病性肺高血圧症. 治療学 44:857-860, 2010
10) Tanaka E, Harigai M, Tanaka M et al:Pulmonary hypertension in systemic lupus erythematosus:evaluation of clinical characteristics and response to immunosuppressive treatment. J Rheumatol 29:282-287, 2002
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Part 4 肺高血圧症の治療戦略を探る

P.148 掲載の参考文献
1) Ed. by Simonneau G, Galie N:Updates in pulmonary hypertension. J Am Coll Cardiol 62(25 Suppl), A1-8, D1-128, 2013
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P.162 掲載の参考文献
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3) Bergot E, Sitbon O, Cottin V et al:Current epoprostenol use in patients with severe idiopathic, heritable or anorexigen-associated pulmonary arterial hypertension:data from the French pulmonary hypertension regis-try. Int J Cardiol 172:561-567, 2014
4) 合同研究班(班長:中西宣文):循環器病の診断と診療に関するガイドライン(2011年度合同研究班報告)肺高血圧症治療ガイドライン(2012年改訂版), 2012
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