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西地那非成功治疗孤立性肺动脉高压的系统性硬化症患者:两例病例报告。

Successful treatment with sildenafil in systemic sclerosis patients with isolated pulmonary arterial hypertension: two case reports.

作者信息

Hayakawa Ikuko, Shirasaki Fumiaki, Hirano Takashi, Oishi Naoto, Hasegawa Minoru, Sato Shinichi, Takehara Kazuhiko

机构信息

Department of Dermatology, Kanazawa University Graduate School of Medical Science , 13-1 Takaramachi, Kanazawa, Ishikawa 920-8641, Japan.

出版信息

Rheumatol Int. 2006 Jan;26(3):270-3. doi: 10.1007/s00296-005-0613-y. Epub 2005 May 25.

DOI:10.1007/s00296-005-0613-y
PMID:15915331
Abstract

We describe two systemic sclerosis (SSc) patients with isolated pulmonary arterial hypertension (PAH) who were given treatment with 50 mg oral sildenafil per day. We evaluated the efficacy of oral sildenafil for isolated PAH in SSc patients by direct assessment with cardiac catheterization before and 6 months after the initiation of sildenafil. Right-heart catheterization demonstrated decreased mean pulmonary artery pressure, decreased pulmonary vascular resistance, and increased cardiac output after treatment with sildenafil. Brain natriuretic peptide levels were gradually decreased. The 6-min walking distance was greatly extended. Moreover, the physical conditions of both patients were much improved. We recognized no adverse events. We propose that oral sildenafil may be beneficial as a selective pulmonary vasodilator and as long-term treatment in SSc patients with isolated PAH.

摘要

我们描述了两名患有孤立性肺动脉高压(PAH)的系统性硬化症(SSc)患者,他们接受了每天50毫克口服西地那非的治疗。我们通过在开始使用西地那非之前和之后6个月进行心脏导管插入术直接评估,来评价口服西地那非对SSc患者孤立性PAH的疗效。右心导管插入术显示,使用西地那非治疗后平均肺动脉压降低、肺血管阻力降低且心输出量增加。脑钠肽水平逐渐下降。6分钟步行距离大幅延长。此外,两名患者的身体状况均有显著改善。我们未发现不良事件。我们认为口服西地那非作为一种选择性肺血管扩张剂,对于患有孤立性PAH的SSc患者可能是一种有益的长期治疗药物。

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Rheumatol Int. 2006 Jan;26(3):270-3. doi: 10.1007/s00296-005-0613-y. Epub 2005 May 25.
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Clin Rev Allergy Immunol. 2012 Dec;43(3):292-301. doi: 10.1007/s12016-012-8312-4.
2
Molecular pathogenesis of pulmonary arterial hypertension.肺动脉高压的分子发病机制
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本文引用的文献

1
Sildenafil improved pulmonary hypertension and peripheral blood flow in a patient with scleroderma-associated lung fibrosis and the raynaud phenomenon.西地那非改善了一名患有硬皮病相关肺纤维化和雷诺现象患者的肺动脉高压和外周血流。
Ann Intern Med. 2003 Nov 18;139(10):871-3. doi: 10.7326/0003-4819-139-10-200311180-00030.
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Sildenafil for treatment of lung fibrosis and pulmonary hypertension: a randomised controlled trial.西地那非治疗肺纤维化和肺动脉高压:一项随机对照试验。
Lancet. 2002 Sep 21;360(9337):895-900. doi: 10.1016/S0140-6736(02)11024-5.
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Sildenafil for primary and secondary pulmonary hypertension.
西地那非用于原发性和继发性肺动脉高压。
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The heart and pulmonary vasculature in scleroderma: clinical features and pathobiology.硬皮病中的心脏和肺血管系统:临床特征与病理生物学
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Inhaled prostacyclin and iloprost in severe pulmonary hypertension secondary to lung fibrosis.吸入性前列环素和伊洛前列素治疗继发于肺纤维化的重度肺动脉高压
Am J Respir Crit Care Med. 1999 Aug;160(2):600-7. doi: 10.1164/ajrccm.160.2.9810008.
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Use of sildenafil (Viagra) in patients with cardiovascular disease. Technology and Practice Executive Committee.心血管疾病患者使用西地那非(万艾可)。技术与实践执行委员会。
Circulation. 1999;99(1):168-77. doi: 10.1161/01.cir.99.1.168.
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Pulmonary hypertension in systemic sclerosis: an analysis of 17 patients.系统性硬化症中的肺动脉高压:17例患者的分析
Br J Rheumatol. 1996 Oct;35(10):989-93. doi: 10.1093/rheumatology/35.10.989.
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Pulmonary hypertension in the CREST syndrome variant of systemic sclerosis.系统性硬化症CREST综合征亚型中的肺动脉高压
Arthritis Rheum. 1986 Apr;29(4):515-24. doi: 10.1002/art.1780290409.
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Ca/CaM-stimulated and cGMP-specific phosphodiesterases in vascular and non-vascular tissues.血管组织和非血管组织中钙/钙调蛋白刺激的以及对环鸟苷酸具有特异性的磷酸二酯酶
Adv Exp Med Biol. 1991;308:191-7. doi: 10.1007/978-1-4684-6015-5_15.