Serum progranulin levels are elevated in dermatomyositis patients with acute interstitial lung disease, predicting prognosis.
Serum progranulin levels are elevated in dermatomyositis patients with acute interstitial lung disease, predicting prognosis.
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急性间质性肺部疾病的皮肌炎患者的血清预素蛋白水平升高，预测预后。
DOI：
10.1186/s13075-015-0547-z
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发表时间：
2015-02-10
影响因子：
4.9
通讯作者：
Akashi K
中科院分区：
文献类型：
--
作者：
Tanaka A;Tsukamoto H;Mitoma H;Kiyohara C;Ueda N;Ayano M;Ohta S;Kimoto Y;Akahoshi M;Arinobu Y;Niiro H;Tada Y;Horiuchi T;Akashi K
原颗粒蛋白（PGRN）是一种多效性生长因子，已成为一种免疫调节分子。由于PGRN在皮肌炎（DM）中的作用尚不清楚，我们研究了血清PGRN水平是否与DM患者的疾病活动性和预后相关，特别是在DM合并间质性肺疾病（ILD）的患者中。采用酶联免疫吸附法测定DM （n =57）、急性/亚急性间质性肺炎（A/SIP）： n =17、慢性间质性肺炎（CIP）： n =24、无ILD患者：n =16)、多发性肌炎（PM, n =21，包括6例ILD）和正常健康对照（NHCs, n =60）患者的血清PGRN水平。我们评估了合并ILD的DM患者血清PGRN水平与ILD活动性指标或预后的相关性。DM患者血清PGRN水平显著高于PM患者（P =0.0025）和NHCs患者（P <0.0001）。在DM患者中，A/SIP患者的水平显著高于CIP患者（P <0.0001）或无ILD患者（P =0.0003）。DM合并ILD患者血清PGRN水平与血清铁蛋白（rS =0.77, P <0.0001）、乳酸脱氢酶（rS =0.54, P =0.0003）、c反应蛋白（rS =0.48, P =0.0015）水平显著相关。此外，在DM合并ILD患者中，血清PGRN水平≥200 ng/ml组（67%）的6个月累积生存率显著低于血清PGRN水平<200 ng/ml组（100%）（P =0.0009）。血清PGRN与DM合并ILD的疾病活动性和预后相关。PGRN可能在糖尿病的发病机制中发挥作用，可能是一种有用的生物标志物。
Progranulin (PGRN), a pleiotropic growth factor, has emerged as an immunoregulatory molecule. Because the roles of PGRN in dermatomyositis (DM) are still unknown, we investigated whether serum PGRN levels are associated with disease activity and prognosis in DM patients, particularly in those with DM complicated with interstitial lung disease (ILD). The serum levels of PGRN were measured by enzyme-linked immunosorbent assay in patients with DM (n =57; acute/subacute interstitial pneumonia (A/SIP): n =17, chronic interstitial pneumonia (CIP): n =24, without ILD: n =16), polymyositis (PM, n =21; including 6 with ILD) and normal healthy controls (NHCs, n =60). We assessed the correlation between the serum PGRN levels and the activity indexes of ILD or prognosis in DM patients with ILD. Serum PGRN levels were significantly higher in DM patients than in PM patients (P =0.0025) and in NHCs (P <0.0001). In DM patients, the levels were significantly higher in patients with A/SIP than in those with CIP (P <0.0001) or without ILD (P =0.0003). The serum PGRN levels in DM patients with ILD significantly correlated with serum ferritin (rS =0.77, P <0.0001), lactate dehydrogenase (rS =0.54, P =0.0003) and C-reactive protein (rS =0.48, P =0.0015) levels. Moreover, in DM patients with ILD, the cumulative survival rate for 6 months was significantly lower in the group with serum PGRN levels ≥200 ng/ml (67%) than in the group with serum PGRN levels <200 ng/ml (100%) (P =0.0009). Serum PGRN is associated with disease activity and prognosis of DM with ILD. PGRN may play a role in the pathogenesis of DM and could be a useful biomarker.
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影响因子：
4
作者：
Bateman, Andrew;Bennett, Hugh P. J.
通讯作者：
Bennett, Hugh P. J.
影响因子：
82.9
作者：
He, ZH;Ong, CHP;Bateman, A
通讯作者：
Bateman, A
影响因子：
3.3
作者：
Haddad, F;Zaldivar, F;Adams, GR
通讯作者：
Adams, GR
影响因子：
9.6
作者：
Connors, Geoffrey R.;Christopher-Stine, Lisa;Danoff, Sonye K.
通讯作者：
Danoff, Sonye K.
影响因子：
15.9
作者：
Kessenbrock, Kai;Froehlich, Leopold;Jenne, Dieter E.
通讯作者：
Jenne, Dieter E.