Jorens P G, Van Marck E, Snoeckx A, Parizel P M
Department of Critical Care Medicine, ZA, Antwerp University Hospital, University of Antwerp, Edegem, Belgium.
Eur Respir J. 2009 Aug;34(2):452-74. doi: 10.1183/09031936.00141708.
Nonthrombotic pulmonary embolism (NTPE) is defined as embolisation to the pulmonary circulation of different cell types (adipocytes, haematopoietic, amniotic, trophoblastic or tumour), bacteria, fungi, foreign material or gas. The purpose of this article is to describe the clinical signs, pathogenesis, diagnosis and treatment of the different NTPE subtypes. The complex and diverse pathogenesis of different subtypes of emboli is subject to continuing speculation and is certainly far more complex than "simple" mechanical obstruction after embolisation of vascular thrombi. Nonthrombotic emboli may also lead to a severe inflammatory reaction both in the systemic and pulmonary circulation, as well as in the lung. NTPE presents a formidable diagnostic challenge, as the condition often presents with very unusual and peculiar clinical signs that are frequently overlooked. They range from very dramatic acute presentations such as acute respiratory distress syndrome to signs observed late in the disease course. Pathological observations play a key role in the exact diagnosis, and sometimes carefully aspirated blood from the pulmonary artery or specific staining of cells recovered from bronchoalveolar lavage fluid may be helpful. Frequently, lung biopsies revealing severe granulomatous reaction or unfortunate post-mortem pathological investigations of pulmonary tissue are necessary to confirm the diagnosis. Here, we also aim to familiarise the reader with the atypical radiological features of NTPE. Thin-section computed tomography of the lungs showing peculiar radiographic findings, such as a feeding vessel, the so-called tree-in-bud pattern or the appearance of micronodules distributed at the termination of bronchovascular bundles, may be observed in certain forms of NTPE. Increased awareness of NTPE as an underestimated cause of acute and chronic embolism, which may result in acute and chronic pulmonary hypertension, is needed. Despite the fact that detailed descriptions of several forms of NTPE have existed for nearly 100 years, well-designed trials have never been performed to evaluate therapy in the different subsets of these patients.
非血栓性肺栓塞(NTPE)定义为不同细胞类型(脂肪细胞、造血细胞、羊水、滋养层细胞或肿瘤细胞)、细菌、真菌、异物或气体栓塞至肺循环。本文旨在描述不同NTPE亚型的临床体征、发病机制、诊断和治疗。不同亚型栓子的复杂多样的发病机制仍在不断探讨中,其肯定远比血管血栓栓塞后的“单纯”机械性梗阻复杂得多。非血栓性栓子还可能在体循环和肺循环以及肺部引发严重的炎症反应。NTPE带来了巨大的诊断挑战,因为该病症常表现出非常不寻常和奇特的临床体征,这些体征经常被忽视。其范围从非常严重的急性表现,如急性呼吸窘迫综合征,到疾病后期观察到的体征。病理观察在准确诊断中起关键作用,有时从肺动脉小心抽取的血液或支气管肺泡灌洗回收细胞的特异性染色可能会有所帮助。通常,需要肺活检显示严重的肉芽肿反应或不幸的是对肺组织进行尸检病理检查来确诊。在此,我们还旨在让读者熟悉NTPE的非典型放射学特征。在某些形式的NTPE中,可能会观察到肺部薄层计算机断层扫描显示出奇特的影像学表现,如供血血管、所谓的树芽征或分布在支气管血管束末端的微小结节。需要提高对NTPE作为急性和慢性栓塞的一个被低估原因的认识,其可能导致急性和慢性肺动脉高压。尽管对几种形式的NTPE已有近100年的详细描述,但从未进行过精心设计的试验来评估这些患者不同亚组的治疗方法。