Regional medical center for National institute of respiratory diseases, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Department of Radiology, Sir Run Run Shaw Hospital, Zhejiang University School of Medicine, Hangzhou, China.
Medicine (Baltimore). 2022 Jul 15;101(28):e29412. doi: 10.1097/MD.0000000000029412.
PTTM is a rare but fatal disease, characterized by endothelial intimal proliferation and pulmonary hypertension due to micro-vascular remodeling. In view of the poor prognosis, new effective strategies are urgently required.
A 51-year-old woman was admitted to hospital for acute progressive dyspnea and dry cough. Clinical tests revealed hypercoagulable state and signs of severe pulmonary hypertension, without evidence of pulmonary embolism on contrast-enhanced CT. CT showed interlobular septal thickening and diffuse ground-glass opacity. Lung perfusion scan indicated multiple segment defect. Further right heart catherization proved a significant increase in pulmonary vascular resistance.
A combination therapy of apatinib and selexipag was administered for treatment of PTTM. The conventional therapies of ventilation, anticoagulation and diuretic medicines were initiated after admission.
Symptoms of PTTM were ameliorated with a reduction in pulmonary artery pressure. The resolution of interlobular septal thickening and ground-glass opacity on CT constituted the clinical benefits from treatment.
Patient with PTTM will benefit from the combination strategy of apatinib, a VEGF-receptor antagonist, and selexipag, an oral prostacyclin receptor agonist.
PTTM 是一种罕见但致命的疾病，其特征是内皮细胞内膜增生和肺高血压，由微血管重构引起。鉴于其预后较差，迫切需要新的有效治疗策略。
一名 51 岁女性因急性进行性呼吸困难和干咳入院。临床检查显示高凝状态和严重肺高血压的迹象，增强 CT 无肺栓塞证据。CT 显示小叶间隔增厚和弥漫性磨玻璃影。肺灌注扫描显示多个节段缺损。进一步的右心导管检查证实肺血管阻力显著增加。
给予阿帕替尼和塞来昔帕联合治疗 PTTM。患者入院后接受了通气、抗凝和利尿剂等常规治疗。
PTTM 的症状得到改善，肺动脉压降低。CT 上小叶间隔增厚和磨玻璃影的消退构成了治疗的临床获益。
PTTM 患者将从 VEGF 受体拮抗剂阿帕替尼和口服前列环素受体激动剂塞来昔帕联合治疗策略中获益。
