French Reference Center for Lupus, Hôpital Pitié-Salpêtrière, APHP, University of Paris VI Pierre and Marie Curie, Paris.
Eur Respir J. 2013 Nov;42(5):1271-82. doi: 10.1183/09031936.00156312. Epub 2013 Feb 8.
Antisynthetase syndrome is characterised by the association of interstitial lung disease and myositis with different anti-tRNA-synthetase antibodies. The occurrence, aetiology and prognosis of pulmonary hypertension have not yet been evaluated. Among 203 consecutive patients, transthoracic echocardiogram and right heart catheterisation results were retrospectively analysed in the light of clinico-biological, morphological and functional parameters. Definitions of pulmonary hypertension were based on the European Society of Cardiology/European Respiratory Society 2009 guidelines, with severe pulmonary hypertension being defined by a mean pulmonary arterial pressure >35 mmHg. Pulmonary hypertension was suspected by transthoracic echocardiogram in 47 (23.2%) cases, corresponding to pulmonary hypertension "possible" (n=27, 13.3%) or "likely" (n=20, 9.9%). Right heart catheterisation was performed in 21 patients, excluding pulmonary hypertension in five and confirming pre-capillary pulmonary hypertension in 16 (7.9%). Although related to interstitial lung disease in all cases, pre-capillary pulmonary hypertension was severe in 13 (81.3%) patients (mean ± sd pulmonary arterial pressure 46 ± 9 mmHg), frequently associated with low cardiac index (mean ± sd 2.3 ± 0.8 L · min(-1) · m(-2)) and high forced vital capacity/diffusing capacity of the lung for carbon monoxide ratio (2.5 ± 0.6). Pulmonary hypertension was significantly associated with a lower survival rate (p<0.001), with a 3-year survival rate of 58%. The occurrence of pulmonary hypertension in antisynthetase syndrome is significant and dramatically worsens the prognosis. Although systematically associated with interstitial lung disease, pulmonary hypertension was usually severe, suggesting a specific pulmonary vascular involvement.
抗合成酶综合征的特征是间质性肺疾病和肌炎与不同的抗 tRNA 合成酶抗体相关。肺动脉高压的发生、病因和预后尚未得到评估。在 203 例连续患者中，回顾性分析了经胸超声心动图和右心导管检查结果，并结合临床生物学、形态学和功能参数进行分析。肺动脉高压的定义基于欧洲心脏病学会/欧洲呼吸学会 2009 年指南，平均肺动脉压>35mmHg 定义为重度肺动脉高压。经胸超声心动图怀疑肺动脉高压 47 例（23.2%），对应肺动脉高压“可能”（n=27，13.3%）或“很可能”（n=20，9.9%）。21 例行右心导管检查，排除 5 例肺动脉高压，证实 16 例毛细血管前性肺动脉高压（7.9%）。尽管所有病例均与间质性肺疾病相关，但 13 例（81.3%）患者的毛细血管前性肺动脉高压严重（平均肺动脉压±标准差 46±9mmHg），常伴有低心指数（平均±标准差 2.3±0.8L·min(-1)·m(-2))和高用力肺活量/一氧化碳弥散量比值（2.5±0.6）。肺动脉高压与生存率显著相关（p<0.001），3 年生存率为 58%。抗合成酶综合征中肺动脉高压的发生率显著，且显著恶化预后。尽管肺动脉高压通常与间质性肺疾病相关，但通常较为严重，提示存在特定的肺血管受累。
