Department of Rheumatology and Immunology, The Affiliated Drum Tower Hospital of Nanjing University Medical School, 321 Zhongshan Road, Nanjing, Jiangsu Province, 210008, China.
Department of Rheumatology and Immunology, Suzhou Wuzhong People's Hospital, 61 Dongwu North Road, Suzhou, Jiangsu Province, 215128, China.
BMC Pulm Med. 2023 Aug 14;23(1):299. doi: 10.1186/s12890-023-02597-z.
Pulmonary arterial hypertension (PAH) is a severe complication of mixed connective tissue disease (MCTD) and contributes to increased morbidity and mortality. Still, the demographic characteristics and risk factors of PAH in MCTD remain poorly understood. This study explored risk factors for PAH development in MCTD.
Data from patients with MCTD and PAH hospitalized from May 2009 to December 2022 in a single center were collected and compared with patients with MCTD without PAH. The variables were analyzed by logistic regression to identify the factors associated with PAH in patients with MCTD. The receiver-operating characteristic (ROC) curve was used to assess the diagnostic value of the identified factors.
Finally, 119 patients with MCTD were included; 46 had PAH. The mean age at PAH onset and diagnosis was 38.9 ± 13.4 and 39.9 ± 13.7 years, respectively. The median pulmonary arterial systolic pressure (PASP) was 67.0 mmHg. The median brain natriuretic peptide (BNP) level was 180.0 pg/ml at PAH diagnosis. Red cell distribution width (RDW) (OR: 2.128; 95% confidence interval: 1.497-3.026; P < 0.001) was associated with PAH in patients with MCTD. There was a positive correlation between RDW and PASP (r = 0.716, P < 0.001). At a cutoff of 15.2%, RDW had the best sensitivity (80.4%) and specificity (82.2%) for PAH.
RDW may serve as a sensitive index to predict PAH in patients with MCTD.
肺动脉高压（PAH）是混合性结缔组织病（MCTD）的严重并发症，增加了发病率和死亡率。然而，MCTD 中 PAH 的人口统计学特征和危险因素仍知之甚少。本研究探讨了 MCTD 中 PAH 发展的危险因素。
收集了 2009 年 5 月至 2022 年 12 月在一家单中心医院住院的 MCTD 合并 PAH 患者和单纯 MCTD 患者的数据，并进行比较。采用 logistic 回归分析变量，以确定与 MCTD 患者 PAH 相关的因素。使用受试者工作特征（ROC）曲线评估确定因素的诊断价值。
最终纳入 119 例 MCTD 患者，其中 46 例合并 PAH。PAH 发病和诊断时的平均年龄分别为 38.9±13.4 岁和 39.9±13.7 岁。平均肺动脉收缩压（PASP）为 67.0mmHg。PAH 诊断时脑钠肽（BNP）水平的中位数为 180.0pg/ml。红细胞分布宽度（RDW）（OR：2.128；95%置信区间：1.497-3.026；P<0.001）与 MCTD 患者的 PAH 相关。RDW 与 PASP 呈正相关（r=0.716，P<0.001）。在截断值为 15.2%时，RDW 对 PAH 的最佳灵敏度（80.4%）和特异性（82.2%）。
RDW 可能是预测 MCTD 患者 PAH 的敏感指标。
