OBJECTIVE: To document changes in pulmonary arterial systolic pressure (PASP) in patients with SLE who have received CYC for any indication. METHODS: Twenty-four patients with SLE pulmonary arterial hypertension (PAH) with a PASP of >30 mmHg by transthoracic echocardiography received i.v. CYC (n = 24) or steroids (n = 24) with or without vasodilators (n = 20). Baseline clinical characteristics and PASP were evaluated before and after therapy at 6 months. Responders were defined as those who had a decrease in PASP of >15 mmHg from baseline along with improvement in their New York Heart Association functional class. RESULTS: There were 11 responders (45.83%), with a decrease in mean PASP from 59.33 mmHg at baseline to 43.29 mmHg at the end of 6 months (P < 0.0001). The decrease in mean PASP from 39.75 mmHg at baseline to 34.4 mmHg at the end of 6 months was significant in four patients who received immunosuppression alone (P = 0.04). There was no difference in baseline PASP and disease activity between responders and non-responders. Two deaths were noted. CONCLUSION: Immunosuppression and vasodilators produced significant improvement in SLE PAH over 6 months.
OBJECTIVE: To document changes in pulmonary arterial systolic pressure (PASP) in patients with SLE who have received CYC for any indication. METHODS: Twenty-four patients with SLE pulmonary arterial hypertension (PAH) with a PASP of >30 mmHg by transthoracic echocardiography received i.v. CYC (n = 24) or steroids (n = 24) with or without vasodilators (n = 20). Baseline clinical characteristics and PASP were evaluated before and after therapy at 6 months. Responders were defined as those who had a decrease in PASP of >15 mmHg from baseline along with improvement in their New York Heart Association functional class. RESULTS: There were 11 responders (45.83%), with a decrease in mean PASP from 59.33 mmHg at baseline to 43.29 mmHg at the end of 6 months (P < 0.0001). The decrease in mean PASP from 39.75 mmHg at baseline to 34.4 mmHg at the end of 6 months was significant in four patients who received immunosuppression alone (P = 0.04). There was no difference in baseline PASP and disease activity between responders and non-responders. Two deaths were noted. CONCLUSION: Immunosuppression and vasodilators produced significant improvement in SLE PAH over 6 months.
Authors: Pedro Anuardo; Monica Verdier; Natali W S Gormezano; Gabriela R V Ferreira; Gabriela N Leal; Alessandro Lianza; Juliana C O A Ferreira; Rosa M R Pereira; Nadia E Aikawa; Maria Teresa Terreri; Claudia S Magalhães; Simone Appenzeller; Maria Carolina Dos Santos; Silvana B Sachetti; Claudio A Len; Gecilmara S Pilleggi; Simone Lotufo; Eloisa Bonfá; Clovis A Silva Journal: Pediatr Cardiol Date: 2016-11-08 Impact factor: 1.655
Authors: Ji Li; Jing-Ran Ma; Zhi-Xing Sun; Jing-Jing Jiang; Yan-Qing Dong; Qian Wang; Hong-Mei Song Journal: Zhongguo Dang Dai Er Ke Za Zhi Date: 2017-06