Idiopathic Pleuroparenchymal Fibroelastosis is Characterized by an Elevated Serum Level of Surfactant Protein-D, but Not Krebs Von Den Lungen-6
Idiopathic Pleuroparenchymal Fibroelastosis is Characterized by an Elevated Serum Level of Surfactant Protein-D, but Not Krebs Von Den Lungen-6
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DOI：
10.1007/s00408-014-9599-0
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发表时间：
2014-10-01
期刊：
影响因子：
5
通讯作者：
Nishioka, Yasuhiko
中科院分区：
文献类型：
--
作者：
Sato, Seidai;Hanibuchi, Masaki;Nishioka, Yasuhiko
特发性胸膜实质纤维弹性组织增生症（IPPFE）是最近报道的一种罕见疾病，其特征是特发性间质性肺炎（IIP）的胸膜和胸膜下实质纤维化增厚，主要发生在上叶。由于这种罕见疾病的临床特征尚未完全阐明，我们研究了IPPFE的临床特征，特别是血清间质生物标志物，表面活性蛋白-D（SP-D）和Krebs von den Lungen-6（KL-6）。所有病例均超过60岁，按体重指数归类为体重不足。所有病例入院时均发现严重限制性通气缺陷。高分辨率计算机断层扫描显示强烈的胸膜增厚与纤维化为主的上叶。在4例病例中，有3例的组织学检查结果也得到证实。有趣的是，所有病例的血清SP-D水平均显著升高，而KL-6在4例病例中的3例中在正常范围内。与特发性肺纤维化和纤维化非特异性间质性肺炎等主要的IIP相比，IPPFE中SP-D水平升高而KL-6水平正常的病例比例明显增高。IPPFE患者SP-D升高的发病机制及临床意义有待进一步研究。
Idiopathic pleuroparenchymal fibroelastosis (IPPFE) is a recently reported rare disease entity characterized by fibrotic thickening of the pleural and subpleural parenchyma predominantly in the upper lobes in idiopathic interstitial pneumonias (IIPs). Because the clinical features of this rare disease are not fully elucidated, we examined the clinical characteristics of IPPFE, especially for serum interstitial biomarkers, surfactant protein-D (SP-D), and Krebs von den Lungen-6 (KL-6).Four consecutive cases of IPPFE who fulfilled the diagnostic criteria were studied. All cases were more than 60 years of age, and were classified as underweight by body mass index. A severe restrictive ventilatory defect was found in all cases on admission. High-resolution computed tomography showed intense pleural thickening associated with fibrosis predominant in upper lobes. Histopathological findings were also confirmed in three out of four cases. Interestingly, the serum level of SP-D was markedly elevated in all cases, while KL-6 was within normal range in three out of four cases. As compared with major IIPs such as idiopathic pulmonary fibrosis and fibrotic nonspecific interstitial pneumonia, IPPFE significantly showed higher frequency of cases with a unique pattern of serum biomarkers, which is characterized by an elevated level of SP-D with a normal range of KL-6.In IPPFE, SP-D might tend to be elevated, while KL-6 was within a normal range. Further study is required to determine the pathogenesis and clinical significance of the elevated SP-D in IPPFE.