Lecomte R, Perrin F, Journeau L, Espitia O, Piriou N, Horeau-Langlard D, Néel A, Masseau A, Hamidou M, Agard C
Service de médecine interne, pôle hospitalo-universitaire 3, centre de compétences maladies systémiques et auto-immunes rares, hôpital Hôtel-Dieu, place Alexis-Ricordeau, 44093 Nantes cedex 1, France.
Clinique cardiologique et des maladies vasculaires, pôle hospitalo-universitaire 2, hôpital Nord Laënnec, boulevard Jacques-Monod, 44093 Nantes cedex 1, France.
Rev Med Interne. 2015 Dec;36(12):794-9. doi: 10.1016/j.revmed.2015.03.013. Epub 2015 Apr 18.
Pulmonary hypertension (PH) may occur in patients with antisynthetase syndrome (ASS) but this association is poorly studied. In this article, we report 4 new cases of PH associated with ASS, and we discuss PH mechanisms in this specific disease.
Four patients (3 females, 1 male) with confirmed ASS associated with anti-Jo1 (n=3), anti-PL7 (n=1), and anti-Ro52 (n=3) antibodies were analyzed. They presented with subacute dyspnea in average ten years after they were first diagnosed as ASS. Diagnosis of pre-capillary PH was made (mean of mPAP: 34mmHg): PAH (n=1), group 3 PH (n=2) and PH associated to hyperthyroidism (n=1). Among three patients who received specific PAH therapy, two had significant improvement in both clinical and hemodynamic parameters.
During ASS, PH may occur in 5 to 10 % of cases, caused by various mechanisms. Unexplained dyspnea may be due to PH among ASS patients.
肺动脉高压（PH）可能发生在抗合成酶综合征（ASS）患者中，但这种关联研究较少。在本文中，我们报告了4例与ASS相关的PH新病例，并讨论了这种特定疾病中PH的机制。
分析了4例确诊为ASS的患者（3例女性，1例男性），其分别与抗Jo1抗体（n = 3）、抗PL7抗体（n = 1）和抗Ro52抗体（n = 3）相关。他们在首次被诊断为ASS后平均10年出现亚急性呼吸困难。确诊为毛细血管前PH（平均肺动脉压：34mmHg）：动脉性肺动脉高压（PAH，n = 1）、3类PH（n = 2）和与甲状腺功能亢进相关的PH（n = 1）。在接受特异性PAH治疗的3例患者中，2例的临床和血流动力学参数均有显著改善。
在ASS期间，5%至10%的病例可能发生PH，由多种机制引起。ASS患者中无法解释的呼吸困难可能归因于PH。
