一个被误诊为间质性肺病的罕见肺动脉高压病例:肺毛细血管血管瘤病。

Q3 Medicine
Tanaffos Pub Date : 2023-03-01
Parisa Adimi, Majid Malekmohammad, Mihan Pourabdollah, Farah Naghashzadeh
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引用次数: 0

摘要

肺毛细血管血管瘤病(PCH)是一种罕见的肺动脉高压病因。我们报告了一名经组织学确诊的肺毛细血管血管瘤病例,患者是一名 42 岁的女士。她在上呼吸道感染后出现进行性呼吸困难和咳嗽。她的白细胞增多,血沉升高,胶原血管化验结果阴性。她的胸部影像学检查显示纵隔淋巴结病变,双侧肺实质玻璃样不张,间质增厚。患者的超声心动图显示右心室严重扩张,肺动脉收缩压约为 105mmHg。右心导管检查显示平均肺动脉压为 30 毫米汞柱,肺毛细血管楔压约为 7 毫米汞柱。开始抗 PH 治疗后,由于患者对抗 PH 治疗的反应异常和放射学检查结果,患者出现了肺水肿。最后,医生对患者进行了开胸肺活检,结果显示其患有肺毛细血管瘤病。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
A Rare Case of Pulmonary Hypertension, Misdiagnosed as Interstitial Lung Disease: Pulmonary Capillary Hemangiomatosis.

Pulmonary capillary hemangiomatosis (PCH) is a rare cause of pulmonary hypertension. We reported a histologically confirmed PCH in a 42-yr-old lady. She presented a progressive dyspnea and cough after an upper respiratory tract infection. She had a leukocytosis and elevated ESR with negative collagen vascular laboratory results. Her chest imaging revealed mediastinal lymphadenopathy with bilateral ground glass opacities with increased interstitial septal thickening in lung parenchyma. Patient echocardiography showed severe right ventricular dilatation with a measured systolic pulmonary arterial pressure of about 105mmHg. Right heart catheterization revealed a mean pulmonary arterial pressure on 30 mmHg with a pulmonary capillary wedge pressure of about 7 mmHg. After starting anti PH treatment, the patient suffered a pulmonary edema and due to abnormal patient response to anti-PH therapies and radiologic findings. Finally, open lung biopsy was performed and showed features of pulmonary capillary hemangiomatosis.

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来源期刊
Tanaffos
Tanaffos Medicine-Critical Care and Intensive Care Medicine
CiteScore
1.10
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