Righi I, Morlacchi L, Rossetti V, Mendogni P, Palleschi A, Tosi D, Pieropan S, Del Gobbo A, Nosotti M
Thoracic Surgery and Lung Transplant Unit, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Department of Pathophysiology and Transplantation, University of Milan, Milan, Italy; Department of Internal Medicine, Respiratory Unit and Adult Cystic Fibrosis Center, Fondazione IRCCS Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.
Transplant Proc. 2019 Jan-Feb;51(1):235-238. doi: 10.1016/j.transproceed.2018.04.071. Epub 2018 Jun 28.
Pleuroparenchymal fibroelastosis (PPFE) is a rare condition, characterized by predominantly upper-lobe pleural and subjacent parenchymal fibrosis, the latter being intra-alveolar with accompanying elastosis of the alveolar walls that leads a clinical progression to respiratory failure. This condition may not be as rare as it seems to be, because nowadays the increasing awareness among specialists is raising the number of new diagnoses. Limited data are available about the prognosis, both for secondary and idiopathic forms. Nevertheless, the idiopathic form seems to be rapidly progressive and no treatment can control the disease, which is why management is challenging. Since the disease was characterized, PPFE cases have been reported in the literature, but most have been secondary rather than idiopathic. Of these, few have successfully undergone lung transplantation as a treatment of end-stage respiratory failure. We here report a successful case of a 38-year-old man affected by idiopathic PPFE who underwent bilateral lung transplantation after extracorporeal membrane oxygenation bridging for an abrupt transition to critical clinical conditions. After a complex postoperative course and a first year characterized by acute rejection, the patient is alive at 5 years with a good quality of life. Our experience confirms that lung transplantation would be a valuable treatment option in case of end-stage idiopathic PPFE cases.
胸膜实质纤维弹性组织增生症（PPFE）是一种罕见疾病，其特征主要为上叶胸膜及相邻实质纤维化，后者为肺泡内纤维化并伴有肺泡壁弹性组织变性，最终导致临床进展为呼吸衰竭。这种疾病可能并不像看起来那么罕见，因为如今专家们的认识不断提高，新诊断病例的数量也在增加。关于继发性和特发性形式的预后，可用数据有限。然而，特发性形式似乎进展迅速，且没有治疗方法能够控制该病，这就是管理具有挑战性的原因。自该疾病被描述以来，文献中已有PPFE病例报道，但大多数为继发性而非特发性。其中，很少有患者成功接受肺移植作为终末期呼吸衰竭的治疗方法。我们在此报告一例成功案例，一名38岁特发性PPFE男性患者，在体外膜肺氧合桥接后因病情突然转变为危急临床状况而接受了双侧肺移植。经过复杂的术后过程以及以急性排斥反应为特征的第一年，患者在5年后仍然存活，生活质量良好。我们的经验证实，对于终末期特发性PPFE病例，肺移植将是一种有价值的治疗选择。
