Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis
Restrictive allograft syndrome post lung transplantation is characterized by pleuroparenchymal fibroelastosis
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DOI：
10.1038/modpathol.2012.171
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发表时间：
2013-03-01
期刊：
影响因子：
7.5
通讯作者：
Hwang, David M.
中科院分区：
文献类型：
--
作者：
Ofek, Efrat;Sato, Masaaki;Hwang, David M.
我们以前曾将限制性同种异体移植物综合征描述为慢性肺移植物功能障碍的一种形式，表现为限制性肺功能下降。然而，限制性同种异体移植物综合征的组织病理学相关性尚未得到令人满意的描述。我们假设，肺胸膜实质纤维弹性组织增生症，如最近在骨髓移植受者中描述的，也可能存在于限制性同种异体移植综合征患者的肺中。回顾性分析了1996年1月1日至2009年6月30日期间接受肺移植的493例患者。在47例具有限制性同种异体移植物综合征临床特征的患者中，16例有楔形活检、再次移植肺外植体或尸检肺标本可供审查。所有肺均显示不同程度的胸膜纤维化。16例中有15例表现为实质性纤维弹性组织增生，其特征为细胞减少的胶原沉积。保持和增厚下面的肺泡间隔弹性网络。纤维弹性组织增生主要分布于胸膜下，部分病例还显示小叶中心和隔旁分布。在纤维弹性组织增生和未受影响的肺实质之间经常观察到明显的分界，成纤维细胞病灶经常存在于该界面。14例同时伴有闭塞性细支气管炎。另一个常见的发现是弥漫性肺泡损伤的存在（13例），通常在获得的标本中
We previously described restrictive allograft syndrome as a form of chronic lung allograft dysfunction, demonstrating restrictive pulmonary function decline. However, the histopathological correlates of restrictive allograft syndrome have yet to be satisfactorily described. We hypothesized that pulmonary pleuroparenchymal fibroelastosis, as has recently been described in bone marrow transplant recipients, may also be present in the lungs of patients with restrictive allograft syndrome. Retrospective review of 493 patients who underwent lung transplantation between 1 January 1996 and 30 June 2009, was conducted. Out of 47 patients with clinical features of restrictive allograft syndrome, 16 had wedge biopsy, re-transplant lung explant, or autopsy lung specimens available for review. All lungs showed varying degrees of pleural fibrosis. Fifteen of 16 showed parenchymal fibroelastosis, characterized by hypocellular collagen deposition with. preservation and thickening of the underlying alveolar septal elastic network. The fibroelastosis was predominantly subpleural in distribution, with some cases also showing centrilobular and paraseptal distribution. A sharp demarcation was often seen between areas of fibroelastosis and unaffected lung parenchyma, with fibroblastic foci often present at this interface. Concurrent features of obliterative bronchiolitis were present in 14 cases. Another common finding was the presence of diffuse alveolar damage (13 cases), usually in specimens obtained