Carbone Roberto G, Monselise Assaf, Barisione Emanuela, Fontana Vincenzo, Paredi Paolo, Puppo Francesco
Department of Internal Medicine, University of Genoa, Genoa, Italy.
Clalit Health Services, Tel Aviv, Israel.
Intern Emerg Med. 2023 Jun;18(4):1087-1093. doi: 10.1007/s11739-023-03267-y. Epub 2023 Apr 17.
Retrospective study comparing pulmonary hypertension risk in systemic sclerosis (SSc) and non-SSc interstitial lung disease patients with usual interstitial pneumonia (UIP) and non-specific interstitial pneumonia (NSIP). Retrospective analysis of 144 interstitial lung disease patients, 53 SSc (32 UIP and 21 NSIP) and 91 non-SSc (47 UIP and 44 NSIP). Pulmonary hypertension was diagnosed as pulmonary systolic artery pressure (PAPs) > 25 mmHg. All SSc and non-SSc patients with pulmonary hypertension were classified WHO Group 3. Pulmonary hypertension was identified in 21/32 (65.6%), 9/21 (42.8%), 14/47 (29.7%), and 28/44 (45.4%) SSc-UIP, SSc-NSIP, control-UIP, and control-NSIP groups, respectively. PAPs mean of SSc-UIP group was higher than control-UIP group (32.6 ± 9.8 vs 28.5 ± 6.6, p-value = 0.02). PAPs mean of SSc-NSIP group was lower than control-NSIP group (27.0 ± 7.1 vs 33.9 ± 8.8, p = 0.002). Frequency of patients with PAP > 25 mmHg in SSc-UIP group was 60% higher in comparison to control-UIP (OR = 1.62, 95% CI 0.51-5.16) and SSc-NSIP (OR = 1.60, 95% CI 0.45-5.70) groups. Logistic regression analysis estimating the linear trend per ten-unit increase in PAPs levels demonstrated an increment for the SSc-UIP group compared to the control-UIP (OR = 2.64, 95% CI 1.25-5.58, p = 0.01) and the control-NSIP (OR = 6.34, 95% CI 2.82-14.3, p < 0.001) groups. The case-control study confirms that pulmonary hypertension is frequently found in SSc patients and demonstrates, for the first time, a significant increased risk of pulmonary hypertension among SSc-UIP patients.
一项回顾性研究，比较系统性硬化症（SSc）和非SSc间质性肺疾病患者中伴有普通型间质性肺炎（UIP）和非特异性间质性肺炎（NSIP）的肺动脉高压风险。对144例间质性肺疾病患者进行回顾性分析，其中53例为SSc患者（32例UIP和21例NSIP），91例为非SSc患者（47例UIP和44例NSIP）。肺动脉高压诊断为肺动脉收缩压（PAPs）>25 mmHg。所有患有肺动脉高压的SSc和非SSc患者均被归类为WHO第3组。在SSc-UIP、SSc-NSIP、对照-UIP和对照-NSIP组中，分别有21/32（65.6%）、9/21（42.8%）、14/47（29.7%）和28/44（45.4%）的患者被发现患有肺动脉高压。SSc-UIP组的PAPs平均值高于对照-UIP组（32.6±9.8 vs 28.5±6.6，p值=0.02）。SSc-NSIP组的PAPs平均值低于对照-NSIP组（27.0±7.1 vs 33.9±8.8，p=0.002）。与对照-UIP组（OR=1.62，95%CI 0.51-5.16）和SSc-NSIP组（OR=1.60，95%CI 0.45-5.70）相比，SSc-UIP组中PAP>25 mmHg的患者频率高出60%。逻辑回归分析估计PAPs水平每增加十个单位的线性趋势，结果显示与对照-UIP组（OR=2.64，95%CI 1.25-5.58，p=0.01）和对照-NSIP组（OR=6.34，95%CI 2.82-14.3，p<0.001）相比，SSc-UIP组呈增加趋势。这项病例对照研究证实，肺动脉高压在SSc患者中很常见，并且首次表明SSc-UIP患者中肺动脉高压风险显著增加。
