Al Otair Hadil Ak, Idrees Majdy M, Saleemi Sarfraz A, Eltoukhy Ahmed M, Alhijji Ali A, Al Habeeb Waleed A, Omair Mohammed A
Department of Critical Care, King Saud University, Riyadh, Saudi Arabia.
Department of Medicine, Division of Pulmonology, Prince Sultan Military Medical City, Riyadh, Saudi Arabia.
Ann Thorac Med. 2019 Jan-Mar;14(1):83-89. doi: 10.4103/atm.ATM_33_18.
Pulmonary arterial hypertension (PAH) is a major cause of morbidity and mortality in patients with systemic sclerosis (SSc). The objective of this study is to describe the clinical characteristics, mortality, and predictors of SSc-PAH in Saudi patients.
Retrospective chart review study of SSc patients who were followed for at least 1 year in three tertiary care centers in Saudi Arabia was conducted. Clinical information, echocardiographic findings, and right heart catheterization (RHC) results were collected. Descriptive statistics were used for demographic and disease characteristics.
Fifty-seven patients with SSc were reviewed. PAH was confirmed by RHC in 40 patients (87.5%, females). Their mean age was 45.43 ± 13.48 years. The mean pulmonary artery pressure was 42.9 ± 12.7 mmHg, the pulmonary vascular resistance index was 19.4 ± 7.7 woods unit, and cardiac index was 2.43 ± 0.68 min/m. The median time from symptoms to first assessment was 42.8 ± 115.62 months. Most patients (77.5%) presented with functional Class III or IV and more than half (22.55%) were on dual combination therapy. Ten patients (25%) SSc PAH died over a follow up period of 37 ± 7 months. Compared to SSc patients without PAH, SSc-PAH patients had shorter 6-min walk distance (6MWD) (296.1 ± 116.5 vs. 399.59 ± 40.60 m, < 0.0001), higher pro-brain natriuretic peptide (1755.8 ± 2123.4 vs. 69.8 ± 44.3 pg/ml = 0.004), and more frequent Raynaud's phenomenon (RP) (90% vs. 35%, < 0.0001). Logistic regression showed RP (odds ratio [OR] =48.58, 95% confidence interval [CI]; 3.73-633.10) and 6MWD (OR 1.02: 95% CI; 1.01-1.03) were associated with the development of PAH.
Our cohort of Saudi SSc-PAH patients has a younger disease onset and a lower mortality than what is described worldwide despite late presentation and requirement of combination therapy. The presence of RP and lower were associated with the development of SSc-PAH.
肺动脉高压（PAH）是系统性硬化症（SSc）患者发病和死亡的主要原因。本研究的目的是描述沙特患者中SSc-PAH的临床特征、死亡率及预测因素。
对在沙特阿拉伯三个三级医疗中心随访至少1年的SSc患者进行回顾性病历审查研究。收集临床信息、超声心动图检查结果及右心导管检查（RHC）结果。采用描述性统计分析人口统计学和疾病特征。
共审查了57例SSc患者。40例患者（87.5%为女性）经RHC确诊为PAH。他们的平均年龄为45.43±13.48岁。平均肺动脉压为42.9±12.7 mmHg，肺血管阻力指数为19.4±7.7伍兹单位，心脏指数为2.43±0.68 min/m。从症状出现到首次评估的中位时间为42.8±115.62个月。大多数患者（77.5%）表现为功能分级III级或IV级，超过一半（22.55%）接受双联联合治疗。10例（25%）SSc PAH患者在37±7个月的随访期内死亡。与无PAH的SSc患者相比，SSc-PAH患者的6分钟步行距离（6MWD）更短（296.1±116.5 vs. 399.59±40.60 m，<0.0001），脑钠肽前体水平更高（1755.8±2123.4 vs. 69.8±44.3 pg/ml，=0.004），雷诺现象（RP）更常见（90% vs. 35%，<0.0001）。逻辑回归分析显示，RP（比值比[OR]=48.58，95%置信区间[CI]：3.73-633.10）和6MWD（OR 1.02：95% CI：1.01-1.03）与PAH的发生相关。
我们的沙特SSc-PAH患者队列发病年龄较轻，尽管就诊较晚且需要联合治疗，但死亡率低于全球报道水平。RP的存在及较低的6MWD与SSc-PAH的发生相关。
