Lyle Pamela L, Weber Robert D, Bogarin Javier, Kircher Tobias
Penrose Hospital, Pathology, 2222 North Nevada Avenue, Colorado Springs, Colorado, 80907, USA.
BMJ Case Rep. 2009;2009. doi: 10.1136/bcr.06.2008.0095. Epub 2009 May 13.
Whipple disease is a rare multisystemic disorder of infectious aetiology caused by Tropheryma whipplei. Pulmonary hypertension is a rare association for which the underlying pathophysiological mechanism is unclear. Our patient was a 54-year-old man with a 1-year history of progressive polyarticular arthritis, and worsening respiratory and gastrointestinal symptoms. Pulmonary artery catheterisation demonstrated moderate-to-severe pulmonary hypertension. Duodenal biopsies, with electron microscopy, were diagnostic of Whipple disease. Involvement by Whipple disease was also evident in the stomach, bone marrow and pulmonary pleura. A 2-week course of intravenous ceftriaxone was initiated and this was followed by a 1-year course of trimethoprim/sulfamethoxazole (160/800), once daily. Nine months into antibiotic treatment, a repeat echocardiogram showed normalisation of the size and function of the cardiac chambers, including the right atrium and right ventricle. There was complete resolution of the severe tricuspid insufficiency and pulmonary hypertension. Whipple disease is not generally considered as a possible cause of pulmonary hypertension but such awareness is important given that it may be potentially reversible with antibiotic therapy.
惠普尔病是一种由惠普尔嗜组织菌引起的罕见的多系统感染性疾病。肺动脉高压是一种罕见的关联情况，其潜在的病理生理机制尚不清楚。我们的患者是一名54岁男性，有1年进行性多关节关节炎病史，呼吸和胃肠道症状不断加重。肺动脉导管检查显示为中度至重度肺动脉高压。十二指肠活检及电子显微镜检查确诊为惠普尔病。胃、骨髓和肺胸膜也明显有惠普尔病累及。开始了为期2周的静脉注射头孢曲松治疗，随后是为期1年的甲氧苄啶/磺胺甲恶唑（160/800）每日一次的治疗。抗生素治疗9个月后，重复超声心动图显示心脏各腔室，包括右心房和右心室的大小和功能恢复正常。严重的三尖瓣关闭不全和肺动脉高压完全消退。惠普尔病一般不被认为是肺动脉高压的可能病因，但鉴于其可能通过抗生素治疗逆转，这种认识很重要。
