Clinical effect of sildenafil combined with bosentan in treatment of connective tissue disease associated moderate-severe pulmonary arterial hypertension_Chinese Journal of Respiratory and Critical Care Medicine

Authors：

ZHU Jianyong ¹ ,  ZENG Yuqin ² , HU Qing ² , XU Ping ³

1. Department of Respiratory Medicine, Renmin Hospital Affiliated to Hubei University of Medicine, Shiyan, Hubei 442000, P. R. China;
2. Department of Endocrine and Rheumatism Diseases, Taihe Hospital Affiliated to Hubei University of Medicine, Shiyan, Hubei 442000, P. R. China;
3. Department of Rheumatism Diseases, Renmin Hospital Affiliated to Hubei University of Medicine, Shiyan, Hubei 442000, P. R. China;

Corresponding author：

ZENG Yuqin, Email: dzjy1973@163.com

Keywords：

Pulmonary arterial hypertension; Connective tissue disease; Sildenafil; Bosentan

DOI：

10.7507/1671-6205.201711045

Video：

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Objective To explore the clinical effect and safety of sildenafil combined with bosentan in the treatment of connective tissue disease associated moderate-severe pulmonary arterial hypertension (CTD-MS-PAH ). Methods Seventy-six patients with CTD-MS-PAH during January 2013 to January 2017 were collected and divided into group A (41 cases) and group B (35 cases) using a stratified random sampling approach. The patients in group A received 25 mg sildenafil tablet therapy, three times a day. The patients group B received 25 mg sildenafil and 62.5 mg bosentan tablet therapy, twice a day. Both groups were treated for 12 weeks. Before and after the trial, all patients undertook six-minute walk test. Meanwhile the Borg dyspnea index score, the pulmonary artery systolic pressure (PASP), right ventricular diameter (RVD), B-type natriuretic peptide (BNP), the partial pressure of oxygen in artery (PaO₂), blood pressure, heart rate, liver and kidney function were all measured. Results After the therapy, six-minute walking distance increased, Borg dyspnea index score decreased, PASP, RVD and plasma BNP decreased, and PaO₂ increased in both groups (all P<0.05), but group B was superior to group A (allP<0.05). There were no significant differences in blood pressure, heart rate, liver or kidney function compared with those before the treatment in both groups (allP>0.05). Conclusion Sildenafil combined with bosentan can significantly decrease the level of pulmonary arterial pressure and effectively improve the cardiopulmonary function in CTD-MS-PAH patients with good safety.

Citation： ZHU Jianyong, ZENG Yuqin, HU Qing, XU Ping. Clinical effect of sildenafil combined with bosentan in treatment of connective tissue disease associated moderate-severe pulmonary arterial hypertension. Chinese Journal of Respiratory and Critical Care Medicine, 2018, 17(4): 369-372. doi: 10.7507/1671-6205.201711045 Copy

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2.	McLaughlin V, Channick RN, Ghofrani HA, et al. Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension. Eur Respir J, 2015, 46(2): 405-413.
3.	孔祥萦, 陆凤霞, 周洁, 等. 西地那非联合波生坦治疗儿童先心病合并肺动脉高压的临床研究. 南京医科大学学报(自然科学版), 2017, 37(7): 861-864.
4.	Dabiri G, Falanga V. Connective tissue ulcers. J Tissue Viability, 2013, 22(4): 92-102.
5.	钱凤文, 陈利明. 超声心动图新技术在评价肺动脉高压患者右心功能中的应用进展. 中国呼吸与危重监护杂志, 2013, 12(4): 427-429.
6.	Roberts JD, Forfia PR. Diagnosis and assessment of pulmonary vascular disease by Doppler echocardiography . Pulm Circ, 2011, 1(2): 160-181.
7.	顾睛, 何建国. 2009年欧美肺动脉高压指南解读与比较.中华医学杂志, 2010, 90(20): 1378-1380.
8.	Fishman AP. Primary pulmonary arterial hypertension: a look back. J Am Coll Cardiol, 2004, 43(12): 2S-4S.
9.	Butrous G. The role of phosphodiesterase inhibitors in the management of pulmonary vascular diseases. Glob Cardiol Sci Pract , 2014, 2014(3): 257-290.
10.	Lajoie AC, Bonnet S, Provencher S. Combination therapy in pulmonary arterial hypertension: recent accomplishments and future challenges. Pulm Circ, 2017, 7(2): 312-325.
11.	曾小峰. 结缔组织病相关肺动脉高压-风湿病的新挑战. 中华风湿病学杂志, 2010, 14(2): 73-75.
12.	张成, 何建国, 卢献灵, 等. 口服西地那非治疗肺动脉高压的效果和安全性初步研究. 中华医学杂志, 2011, 91(6): 370-374.
13.	Wang RC, Jiang FM, Zheng QL, et al. Efficacy and safety of sildenafil treatment in pulmonary arterial hypertension: a systemaic review. Respir Med, 2014, 108(3): 531-537.
14.	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, 2012, 142(2): 448-456.
15.	Sompradeekul S, Wattanasiriphakdee S. Hemodynamic effect of iloprost inhalation and oral sildenafil during acute vasoreactivity test in pulmonary arterial hypertension. J Med Assoc Thai, 2015, 98(2): 144-149.
16.	Baptista R, Castro G, Dasilva AM, et al. Long-term effect of bosentan in pulmonary hypertension associated with complex congenital heart disease. Rev Port Cardiol, 2013, 32(2): 123-129.

1. 张缪佳. 加强对结缔组织病相关肺动脉高压的预后评估. 中华风湿病学杂志, 2012, 16(8): 505-507.
2. McLaughlin V, Channick RN, Ghofrani HA, et al. Bosentan added to sildenafil therapy in patients with pulmonary arterial hypertension. Eur Respir J, 2015, 46(2): 405-413.
3. 孔祥萦, 陆凤霞, 周洁, 等. 西地那非联合波生坦治疗儿童先心病合并肺动脉高压的临床研究. 南京医科大学学报(自然科学版), 2017, 37(7): 861-864.
4. Dabiri G, Falanga V. Connective tissue ulcers. J Tissue Viability, 2013, 22(4): 92-102.
5. 钱凤文, 陈利明. 超声心动图新技术在评价肺动脉高压患者右心功能中的应用进展. 中国呼吸与危重监护杂志, 2013, 12(4): 427-429.
6. Roberts JD, Forfia PR. Diagnosis and assessment of pulmonary vascular disease by Doppler echocardiography . Pulm Circ, 2011, 1(2): 160-181.
7. 顾睛, 何建国. 2009年欧美肺动脉高压指南解读与比较.中华医学杂志, 2010, 90(20): 1378-1380.
8. Fishman AP. Primary pulmonary arterial hypertension: a look back. J Am Coll Cardiol, 2004, 43(12): 2S-4S.
9. Butrous G. The role of phosphodiesterase inhibitors in the management of pulmonary vascular diseases. Glob Cardiol Sci Pract , 2014, 2014(3): 257-290.
10. Lajoie AC, Bonnet S, Provencher S. Combination therapy in pulmonary arterial hypertension: recent accomplishments and future challenges. Pulm Circ, 2017, 7(2): 312-325.
11. 曾小峰. 结缔组织病相关肺动脉高压-风湿病的新挑战. 中华风湿病学杂志, 2010, 14(2): 73-75.
12. 张成, 何建国, 卢献灵, 等. 口服西地那非治疗肺动脉高压的效果和安全性初步研究. 中华医学杂志, 2011, 91(6): 370-374.
13. Wang RC, Jiang FM, Zheng QL, et al. Efficacy and safety of sildenafil treatment in pulmonary arterial hypertension: a systemaic review. Respir Med, 2014, 108(3): 531-537.
14. 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, 2012, 142(2): 448-456.
15. Sompradeekul S, Wattanasiriphakdee S. Hemodynamic effect of iloprost inhalation and oral sildenafil during acute vasoreactivity test in pulmonary arterial hypertension. J Med Assoc Thai, 2015, 98(2): 144-149.
16. Baptista R, Castro G, Dasilva AM, et al. Long-term effect of bosentan in pulmonary hypertension associated with complex congenital heart disease. Rev Port Cardiol, 2013, 32(2): 123-129.

Chinese Journal of Respiratory and Critical Care Medicine

Clinical effect of sildenafil combined with bosentan in treatment of connective tissue disease associated moderate-severe pulmonary arterial hypertension

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