Ribeiro Baptista Bruno, Petitpain Nadine, Gomez Emmanuel, Yelehé-Okouma Melissa, Valentin Simon, Guillaumot Anne, Chabot François, Chaouat Ari
Département de pneumologie, CHRU de Nancy, rue du Morvan, 54500, Vandœuvre-lès-Nancy, France.
Centre Régional de Pharmacovigilance, CHRU de Nancy, Hôpital Central, 29, avenue du Maréchal de Lattre de Tassigny, Nancy, France.
Fundam Clin Pharmacol. 2019 Feb;33(1):127-129. doi: 10.1111/fcp.12396. Epub 2018 Jul 15.
4-Aminopyridine (4-AP) is a recent treatment indicated to improve walking in patient with multiple sclerosis. We report the first case of pulmonary arterial hypertension (PAH) that we attribute to the use of 4-AP. A 64-year-old woman with multiple sclerosis presented with dyspnea. After excluding other secondary causes of pulmonary hypertension, a diagnosis of severe PAH due to 4-AP was made based on right heart catheterization. History revealed that the dyspnea began with the initiation of 4-AP. After discontinuation of 4-AP therapy and initiation of ambrisentan and tadalafil, dyspnea and pulmonary arterial pressure have improved significantly and one specific PAH treatment was stopped. 4-AP is an outward rectifying potassium channel blocker with a vasoconstrictor effect in animal's pulmonary artery. According to the chronological sequence of events, the lack of other etiology, and its pharmacological plausibility, 4-AP is highly suspected to have induced our patient's PAH.
4-氨基吡啶（4-AP）是一种近期用于改善多发性硬化症患者行走能力的治疗药物。我们报告了首例归因于使用4-AP的肺动脉高压（PAH）病例。一名64岁的多发性硬化症女性出现呼吸困难。在排除其他肺动脉高压的继发原因后，基于右心导管检查诊断为4-AP所致的重度PAH。病史显示，呼吸困难始于开始使用4-AP后。停用4-AP治疗并开始使用安立生坦和他达拉非后，呼吸困难和肺动脉压力显著改善，一种特定的PAH治疗药物停用。4-AP是一种外向整流钾通道阻滞剂，在动物肺动脉中具有血管收缩作用。根据事件的时间顺序、缺乏其他病因以及其药理学合理性，高度怀疑4-AP诱发了我们患者的PAH。
