Silvia Bocci, Miriana D'Alessandro, Laura Bergantini, Alfonso Cerase, Monica Ulivelli, Paola Rottoli, Dalila Cavallaro, Sara Gangi, Elena Bargagli, Fabio Giannini
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引用次数: 0
Abstract
Background: Krebs von den Lungen-6 (KL-6) is a high molecular weight (MW) glycoprotein mainly secreted by type II pneumocytes because of lung damage or during regeneration. Neurosarcoidosis (NS), where sarcoid granulomas involve the nervous system, occurs in 5-20% of patients with sarcoidosis. No data is currently available on KL-6 in serum or CSF of NS patients. The present study compared KL-6 concentrations in serum and CSF of NS patients versus others with neurodegenerative (ND) or chronic inflammatory demyelinating (DM) diseases.
Materials and methods: Nine NS patients (mean age 46.2 years, range 16-61 years, M/F 5/4), nine patients with a chronic neurodegenerative disease (mean age 53.1 years, range 37-65 years, M/F 5/4) and nine patients with a chronic demyelinating disease (mean age 46.3 years, range 18-65 years, M/F 5/4) were retrospectively enrolled.
Results: Measurable CSF concentrations of KL-6 were detected in 7/9 NS patients but in no ND or DM patients. No significant differences in CSF concentrations of ACE were observed between the three groups (p=0.0819). In NS patients, CSF concentrations of KL-6 were directly correlated with CSF albumin index (r=0.98; p<0.0001), albumin (r=0.979, p=0.0001), IgG (r=0.928, p=0.0009) and total protein concentrations (r=0.945, p=0.0004).
Discussion: KL-6 is a high MW protein, under physiological conditions it is unlikely to cross the blood-brain barrier. We found KL-6 in CSF from NS and not from ND and DM patients. The finding sustains the specificity of changes in KL-6 in this granulomatous disease, suggesting it as a candidate biomarker for recognition of NS.
背景:Krebs von den Lungen-6 (KL-6)是一种高分子量(MW)糖蛋白,主要由II型肺细胞在肺损伤或再生时分泌。神经结节病(NS),结节性肉芽肿累及神经系统,发生在5-20%的结节病患者中。目前没有关于NS患者血清或脑脊液中KL-6的数据。本研究比较了NS患者与其他神经退行性(ND)或慢性炎症性脱髓鞘(DM)疾病患者血清和脑脊液中的KL-6浓度。材料和方法:回顾性研究9例NS患者(平均年龄46.2岁,范围16-61岁,M/F 5/4)、9例慢性神经退行性疾病患者(平均年龄53.1岁,范围37-65岁,M/F 5/4)和9例慢性脱髓鞘疾病患者(平均年龄46.3岁,范围18-65岁,M/F 5/4)。结果:7/9 NS患者可检测到脑脊液中KL-6的浓度,而ND和DM患者未检测到。三组脑脊液中ACE浓度差异无统计学意义(p=0.0819)。NS患者脑脊液中KL-6浓度与脑脊液白蛋白指数直接相关(r=0.98;讨论:KL-6是一种高分子量蛋白,在生理条件下不太可能穿过血脑屏障。我们在NS患者脑脊液中发现KL-6,而在ND和DM患者脑脊液中未发现KL-6。这一发现维持了KL-6在这种肉芽肿性疾病中变化的特异性,表明它是识别NS的候选生物标志物。
期刊介绍:
Sarcoidosis Vasculitis and Diffuse Lung Disease is a quarterly journal founded in 1984 by G. Rizzato. Now directed by R. Baughman (Cincinnati), P. Rottoli (Siena) and S. Tomassetti (Forlì), is the oldest and most prestigious Italian journal in such field.