Department of Research and Development, Inova Diagnostics, San Diego, CA, United States.
Liver Unit, Hospital Clínic, Institut D'Investigacions Biomèdiques August Pi i Sunyer, CIBERehd, University of Barcelona, Barcelona, Spain.
Front Immunol. 2019 Apr 3;10:662. doi: 10.3389/fimmu.2019.00662. eCollection 2019.
Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, is present worldwide. Autoantibodies, in particular anti-mitochondrial antibodies (AMA) detected by indirect immunofluorescence assays or newer solid phase immunoassays can detect most, but not all individuals with PBC. Detection of antibodies to the anti-nuclear antigens sp100 and gp210 can identify additional PBC patients, but some seronegative patients remain, often resulting in delayed diagnosis and treatment. Antibodies to kelch-like 12 (KLHL12) and hexokinase 1 (HK-1) were recently identified as new biomarkers for PBC and notably identify patients who are negative for conventional autoantibodies. To become globally adopted, it is important to validate these new biomarkers in different geographic areas. In the present study we evaluated the prevalence of anti-KLHL12 (measured by a KLHL12-derived peptide referred to as KL-p) and anti-HK-1 antibodies by ELISA at five sites within Europe and North America and demonstrated the presence of these antibodies in patients with PBC in all geographies.
原发性胆汁性胆管炎（PBC），以前称为原发性胆汁性肝硬化，在全球范围内存在。自身抗体，特别是通过间接免疫荧光测定或更新的固相免疫测定检测到的抗线粒体抗体（AMA）可以检测到大多数但不是所有的 PBC 患者。检测针对核抗原 sp100 和 gp210 的抗体可以识别其他 PBC 患者，但仍有一些血清阴性患者，通常导致诊断和治疗延迟。最近发现抗 kelch 样 12（KLHL12）和己糖激酶 1（HK-1）抗体是 PBC 的新生物标志物，并且显著识别出对常规自身抗体阴性的患者。为了在全球范围内得到采用，在不同的地理区域验证这些新的生物标志物非常重要。在本研究中，我们在欧洲和北美五个地点通过 ELISA 评估了抗 KLHL12（通过称为 KL-p 的 KLHL12 衍生肽测量）和抗 HK-1 抗体的流行率，并证明了这些抗体在所有地理区域的 PBC 患者中存在。
