Shimizu Masaki, Hashida Yoko, Ueno Kazuyuki, Yokoyama Tadafumi, Nakayama Yuko, Saito Takekatsu, Ohta Kunio, Takehara Kazuhiko, Yachie Akihiro
Department of Pediatrics, School of Medicine, Institute of Medical, Pharmaceutical and Health Sciences, Kanazawa University, Kanazawa, Japan.
Pediatr Cardiol. 2011 Oct;32(7):1040-2. doi: 10.1007/s00246-011-0056-z. Epub 2011 Jul 26.
Pulmonary arterial hypertension (PAH) when associated with systemic sclerosis (SSc) (SSc-PAH) is one of the leading causes of mortality and is found in 10-15% of adult patients with SSc. The ET receptor antagonist bosentan has been shown to be effective in the treatment of adult patients with SSc-PAH. Furthermore, it has been shown that bosentan ameliorates decreased skin perfusion and digital ulceration secondary to SSc. However, the effectiveness and safety of bosentan for treatment of juvenile SSc still remains unclear. We describe a case of juvenile SSc-PAH successfully treated with bosentan. The present case shows that bosentan ameliorated PAH and peripheral circulation as evaluated by cold stress thermography. No bosentan-related adverse events such as liver dysfunction were observed. Prospective randomized trials are required to validate the effectiveness of bosentan for patients with juvenile SSc; however, bosentan might be useful for the management of patients with juvenile SSc.
肺动脉高压（PAH）与系统性硬化症（SSc）相关时（SSc-PAH）是主要死亡原因之一，在10%-15%的成年SSc患者中存在。内皮素受体拮抗剂波生坦已被证明对治疗成年SSc-PAH患者有效。此外，还表明波生坦可改善继发于SSc的皮肤灌注减少和指端溃疡。然而，波生坦治疗青少年SSc的有效性和安全性仍不清楚。我们描述了一例成功用波生坦治疗的青少年SSc-PAH病例。本病例表明，通过冷应激热成像评估，波生坦改善了PAH和外周循环。未观察到波生坦相关的不良事件，如肝功能障碍。需要进行前瞻性随机试验来验证波生坦对青少年SSc患者的有效性；然而，波生坦可能对青少年SSc患者的管理有用。
