Circulating KL-6 predicts the outcome of rapidly progressive idiopathic pulmonary fibrosis
Circulating KL-6 predicts the outcome of rapidly progressive idiopathic pulmonary fibrosis
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DOI：
10.1164/ajrccm.158.5.9803115
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发表时间：
1998-11-01
影响因子：
24.7
通讯作者：
Hiwada, K
中科院分区：
文献类型：
--
作者：
Yokoyama, A;Kohno, N;Hiwada, K
为了寻找大剂量皮质类固醇（“脉冲疗法”）对快速进展的特发性肺纤维化（IPF）患者治疗效果的早期预测标志物，我们评估了14例这样的患者，他们接受了每周至少3周的脉冲疗法。8名患者对治疗有反应并存活。然而，6例患者无效，所有患者均在治疗后3个月内死亡。检测治疗前、治疗后1周和3周血清KL-6（MUC 1粘蛋白）、中性粒细胞弹性蛋白酶（NE）和乳酸脱氢酶（LDH）水平。KL-6水平在存活的患者中显著降低，而KL-6水平在死亡的患者中倾向于增加。NE值无明显变化。LDH水平在1周时显著下降，存活患者在3周时有下降趋势。然而，在死亡的患者中，它们没有显著变化。在治疗的第一个周期，当临床效果可能不明显时，KL-6而不是LDH水平的降低与有利的结果显著相关，而它们的增加与不良结果相关。结果表明，KL-6监测可能有助于在快速进展的IPF管理中做出替代治疗的早期临床决策。
Searching for early predictive markers of the therapeutic effects of high-dose corticosteroids ("pulse therapy") on patients with rapidly progressing idiopathic pulmonary fibrosis (IPF), we evaluated 14 such patients, who had received weekly pulse therapy for at least 3 wk. Eight patients responded to the treatment and survived. However, six patients failed to respond, and all of them died within 3 mo after treatment. Serum levels of KL-6 (MUC1 mucin), neutrophil elastase (NE), and lactate dehydrogenase (LDH) were measured before, and at 1 wk and 3 wk after treatment. Levels of KL-6 decreased significantly in patients who lived, whereas KL-6 levels tended to increase in patients who died. The values of NE did not change significantly. LDH levels decreased significantly at 1 wk, and tended to decrease at 3 wk in patients who lived. However, in patients who died, they did not significantly change. At the first cycle of treatment when clinical effects may not be evident, the decrease in KL-6 but not LDH levels was significantly related to a favorable outcome, whereas their increase was related to a poor outcome. Results suggest that monitoring with KL-6 may contribute to early clinical decisions for alternative therapy in the, management of rapidly progressing IPF.