Le Houssine P, Karmochkine M, Ledru F, Batisse D, Piketty C, Kazatchkine M D, Weiss L
Service d'immunologie clinique, hôpital européen Georges-Pompidou, 20, rue Leblanc, 75908 Paris, France.
Rev Med Interne. 2001 Dec;22(12):1196-203. doi: 10.1016/s0248-8663(01)00491-x.
In medical literature, primary pulmonary hypertension occurs in 0.5% of human immunodeficiency virus (HIV)-infected patients, irrespective of the stage of the HIV disease, and is more frequent in drug users. Plexogenic arteriopathy is the most frequent histological lesion.
We retrospectively report on nine cases of primary pulmonary hypertension during HIV infection.
The subjects were four women and five men, mean age 38 years old. Four of them had been sexually contaminated and five had contracted the disease through intravenous drug use. At the time primary pulmonary hypertension was diagnosed, mean CD4 cell count was 234 +/- 217/mm3 and the viral load was low or undetectable. Primary pulmonary hypertension has been diagnosed an average of 7 months after the first cardiovascular clinical signs had started. Despite anti-coagulant (7/9 cases), vasodilatator (4/9 cases) and/or diuretic (7/9 cases) therapy, the progression of the disease quickly turned out to be negative (seven deaths).
Diagnosis of primary pulmonary hypertension should be considered when unexplained dyspnea occurs in an HIV-positive patient. At initial evaluation, alterations of hemodynamic parameters are usually less severe than during idiopathic primary pulmonary hypertension, but their progression is quicker and more severe, independent of the patient's immune status. Current data do not allow the determination of whether antiretroviral therapy is active in primary pulmonary hypertension evolution. Therapeutic evaluation with prostacyclin is currently being carried out. While the life expectancy of HIV-infected patients extends, primary pulmonary hypertension occurrence could increase and call for early diagnosis, thus allowing for specific care.
在医学文献中，原发性肺动脉高压在0.5%的人类免疫缺陷病毒（HIV）感染患者中出现，与HIV疾病的阶段无关，且在吸毒者中更为常见。丛状动脉病是最常见的组织学病变。
我们回顾性报告了9例HIV感染期间的原发性肺动脉高压病例。
研究对象为4名女性和5名男性，平均年龄38岁。其中4人通过性接触感染，5人通过静脉吸毒感染。在诊断原发性肺动脉高压时，平均CD4细胞计数为234±217/mm³，病毒载量低或检测不到。原发性肺动脉高压在首次出现心血管临床症状后平均7个月被诊断出来。尽管进行了抗凝治疗（7/9例）、血管扩张剂治疗（4/9例）和/或利尿剂治疗（7/9例），疾病进展很快显示为不良（7例死亡）。
当HIV阳性患者出现无法解释的呼吸困难时，应考虑原发性肺动脉高压的诊断。在初始评估时，血流动力学参数的改变通常比特发性原发性肺动脉高压时轻，但进展更快、更严重，且与患者的免疫状态无关。目前的数据无法确定抗逆转录病毒疗法在原发性肺动脉高压进展中是否有效。目前正在进行前列环素的治疗评估。随着HIV感染患者预期寿命的延长，原发性肺动脉高压的发生率可能增加，需要早期诊断，从而进行针对性治疗。
