A clinical analysis of 13 patients with scleroderma renal crisis and pulmonary arterial hypertension
10.3760/cma.j.cn141217-20210715-00277
- VernacularTitle:硬皮病肾危象合并肺动脉高压13例临床分析
- Author:
Juan LI
1
;
Mengzhu ZHAO
;
Dong XU
;
Jiaxin ZHOU
;
Mengtao LI
;
Xiaofeng ZENG
;
Fengchun ZHANG
;
Yong HOU
Author Information
1. 中国医学科学院 北京协和医学院 北京协和医院（风湿免疫病学教育部重点实验室 国家皮肤与免疫疾病临床医学研究中心）风湿免疫科，北京 100730
- Keywords:
Scleroderma, systemic;
Hypertension, pulmonary;
Scleroderma renal crisis
- From:
Chinese Journal of Rheumatology
2022;26(4):238-242
- CountryChina
- Language:Chinese
-
Abstract:
Objective:To describe a series of systemic sclerosis (SSc) patients with the combination of scleroderma renal crisis (SRC) and pulmonary arterial hypertension (PAH).Methods:The medical records of 472 SSc patients in Peking Union Medical College Hospital between January 2012 and October 2020 were reviewed and a retrospective analysis of the characteristics of patients with SRC and PAH among SSc patients was conducted.Results:Thirteen patients suffered from SRC and PAH in the SSc patients, 1 case was limited cutaneous SSc, and 12 cases were diffuse cutaneous SSc. Five patients had renal crisis before pulmonary arterial hypertension, 4 patients had pulmonary arterial hypertension before the occurrence of renal crisis, and the remaining 4 patients were found at the same time. Among them, 11 patients had Raynaud's phenomenon, 7 had gastrointestinal bleeding, 6 had pulmonary edema and 3 had telangiectasias. Twelve cases were positive for anti-nuclear antibodies and 4 cases were positive for anti-Scl-70 antibodies. N-terminal pro-brain natriuretic peptide (NT-proBNP)>1 400 ng/L in 11 patients. Two patients had thrombotic microangiopathy (TMA). Among the 13 patients, 3 patients died during hospitalization, 2 patients were lost to follow-up, and 2 patients died within 5 years of follow-up. Six patients survived, and 1 of the 4 patients with regular dialysis were discharged from dialysis.Conclusion:In patients with scleroderma, SRC can occur earlier, later than, or at the same time with SSc-PAH. Patients may have a higher incidence of gastrointestinal bleeding and higher level of NT-proBNP. PDE5i or ERAs may be beneficial.
