Division of Cardiology, Department of Internal Medicine, Ribeirão Preto Medical School.
Department of Internal Medicine, Ribeirão Preto Medical School, University of São Paulo.
Rheumatology (Oxford). 2023 Feb 6;62(SI):SI101-SI106. doi: 10.1093/rheumatology/keac413.
Autologous haematopoietic stem cell transplantation (AHSCT) is a disease-modifying treatment for patients with severe SSc. Here, we aimed at assessing cardiopulmonary function outcomes of SSc patients after AHSCT.
Twenty-seven SSc adult patients treated with AHSCT were included in this retrospective study. Most had the diffuse cutaneous subset (93%) and pulmonary involvement (85%). Before and 12 months after AHSCT, patients underwent cardiopulmonary exercise testing, transthoracic echocardiography, pulmonary function test with diffusing capacity for carbon monoxide (DLCO), 6-min walk test (6MWT) and quality of life evaluations.
After AHSCT, the peak VO2 increased from 954 to 1029 ml/min (P = 0.02), the percentage of predicted peak VO2 increased from 48.9 to 53.5 m (P = 0.01), and the distance measured by the 6MWT increased from 445 to 502 m (P = 0.01), compared with baseline. Improvements in peak VO2 correlated positively with improvements in 6MWT distance, and negatively with a decrease in resting heart rate. At baseline, patients with DLCO >70% had higher peak VO2 values than those with DLCO <70% (P = 0.04), but after AHSCT all patients showed improved VO2 values, regardless of baseline DLCO levels. Increases in VO2 levels after AHSCT positively correlated with increases in the physical component scores of the Short Form-36 quality of life questionnaire (r = 0.70; P = 0.0003).
AHSCT improves the aerobic capacity of SSc patients probably reflecting combined increments in lungs, skeletal muscle and cardiac function.
自体造血干细胞移植（AHSCT）是治疗严重系统性硬化症（SSc）患者的一种疾病修正治疗方法。在此，我们旨在评估 AHSCT 后 SSc 患者的心肺功能结局。
这项回顾性研究纳入了 27 例接受 AHSCT 的 SSc 成年患者。大多数患者为弥漫性皮肤亚型（93%）和肺部受累（85%）。在 AHSCT 前后，患者均接受心肺运动试验、经胸超声心动图、一氧化碳弥散量（DLCO）测定的肺功能检查、6 分钟步行试验（6MWT）和生活质量评估。
AHSCT 后，峰值 VO2 从 954ml/min 增加到 1029ml/min（P=0.02），预测峰值 VO2 的百分比从 48.9%增加到 53.5%（P=0.01），6MWT 测量的距离从 445m 增加到 502m（P=0.01），与基线相比。峰值 VO2 的改善与 6MWT 距离的改善呈正相关，与静息心率的降低呈负相关。在基线时，DLCO＞70%的患者的峰值 VO2 值高于 DLCO＜70%的患者（P=0.04），但在 AHSCT 后，所有患者的 VO2 值均有所提高，而与基线时的 DLCO 水平无关。AHSCT 后 VO2 水平的升高与健康调查简表 36 项生活质量问卷的身体成分评分的升高呈正相关（r=0.70；P=0.0003）。
AHSCT 改善了 SSc 患者的有氧运动能力，这可能反映了肺、骨骼肌和心功能的综合增强。
