Morelli S, Ferri C, Di Francesco L, Baldoncini R, Carlesimo M, Bottoni U, Properzi G, Santucci A
University of Rome La Sapienza, Institute of I Clinica Medica, Andrea Cesalpino Foundation, Rome, Italy.
Ann Rheum Dis. 1995 Sep;54(9):730-4. doi: 10.1136/ard.54.9.730.
To investigate the behaviour of circulating endothelin-1 (ET-1) in patients affected by systemic sclerosis and to elucidate the relationship between systemic and pulmonary plasma peptide and arterial pressure levels.
Plasma ET-1 concentrations were determined in 48 patients affected by systemic sclerosis (41 women, seven men; mean age 47.2 (SD 5.5) years) with or without systemic or pulmonary hypertension (or both). A group of 18 normal volunteers served as controls (15 women, three men; mean age 45.0 (10.1) years).
Plasma ET-1 levels were significantly greater in patients affected by systemic sclerosis (1.65 (0.29) pg/ml) than in controls (0.63 (0.19) pg/ml) (p < 0.0001). Pulmonary artery systolic hypertension alone was present in 14 patients with systemic sclerosis (50.5 (8.49) mm Hg, range 37-67 mm Hg), and systemic hypertension alone (160.7 (5.9)/100.6 (3.2) mm Hg) was present in 11 patients. Both conditions were present in 12 patients, while 11 patients had systemic hypertension. There were no significant differences in plasma ET-1 levels between patients with pulmonary hypertension alone (1.62 (0.21) pg/ml) and those with systemic hypertension alone (1.65 (0.43) pg/ml). In particular, patients with normal pulmonary artery and systemic pressures (n = 11) had plasma ET-1 concentrations identical to those found in patients (n = 12) with both pulmonary and systemic hypertension (1.70 (0.15) v 1.64 (0.35) pg/ml, respectively). No correlations were observed between plasma ET-1 and either pulmonary or systemic pressures.
Systemic sclerosis is characterised by increased plasma ET-1 levels, but neither pulmonary nor systemic hypertension are accompanied by further increase in plasma peptide levels.
研究系统性硬化症患者循环内皮素-1（ET-1）的行为，并阐明全身和肺血浆肽水平与动脉压之间的关系。
测定48例系统性硬化症患者（41例女性，7例男性；平均年龄47.2（标准差5.5）岁）的血浆ET-1浓度，这些患者伴有或不伴有全身或肺动脉高压（或两者兼有）。18名正常志愿者作为对照组（15例女性，3例男性；平均年龄45.0（10.1）岁）。
系统性硬化症患者的血浆ET-1水平（1.65（0.29）pg/ml）显著高于对照组（0.63（0.19）pg/ml）（p<0.0001）。14例系统性硬化症患者仅存在肺动脉收缩期高血压（50.5（8.49）mmHg，范围37-67mmHg），11例患者仅存在系统性高血压（160.7（5.9）/100.6（3.2）mmHg）。12例患者同时存在这两种情况，而11例患者有系统性高血压。仅患有肺动脉高压的患者（1.62（0.21）pg/ml）和仅患有系统性高血压的患者（1.65（0.43）pg/ml）之间的血浆ET-1水平无显著差异。特别是，肺动脉和全身压力正常的患者（n=11）的血浆ET-1浓度与同时患有肺动脉和全身高血压的患者（n=12）相同（分别为1.70（0.15）和1.64（0.35）pg/ml）。未观察到血浆ET-1与肺动脉或全身压力之间的相关性。
系统性硬化症的特征是血浆ET-1水平升高，但肺动脉高压和系统性高血压均未伴有血浆肽水平的进一步升高。
