Gatselis Nikolaos K, Zachou Kalliopi, Lygoura Vasiliki, Azariadis Kalliopi, Arvaniti Pinelopi, Spyrou Elias, Papadamou Georgia, Koukoulis George K, Dalekos George N, Rigopoulou Eirini I
Department of Medicine and Research Laboratory of Internal Medicine, Medical School, University of Thessaly, Larissa, Greece.
Department of Pathology, Medical School, University of Thessaly, Larissa, Greece.
Eur J Intern Med. 2017 Jul;42:81-88. doi: 10.1016/j.ejim.2017.05.006. Epub 2017 May 20.
BACKGROUND & AIMS: Primary biliary cholangitis (PBC) is a disease with rising prevalence and considerable geographical variation. To describe the prevalence, spatial and time distribution, baseline characteristics, response to treatment, outcome and the validity of GLOBE score in a large cohort of Greek PBC patients as an independent validation of this score has not been done so far.
The last 16years, 482 PBC patients (86.5% females) were evaluated and analysed retrospectively, using a prospectively collected database. Special attention was paid to the assessment of treatment response according to GLOBE score.
Age at initial evaluation was 56.3±13.7years. Among 432 Thessaly residents, prevalence was 582/million (non-homogeneous distribution). Nineteen districts showed a prevalence >800/million. Symptomatic disease onset could be identified in 91 patients, with a significant peak during spring (P=0.03). At diagnosis, 43.6% were asymptomatic and 16.2% cirrhotic. Male sex (P=0.02), older age (P<0.001), alcohol consumption (P<0.01) and concomitant liver disease (P<0.001) were negative prognostic factors for cirrhosis. During a median [interquartile range, range] follow-up of 5.1 (7.8, 15.7) years, 62 patients died or underwent liver transplantation. Patients with GLOBE score>0.30 had significantly worse prognosis (P<0.001) with 5-, 10-, and 15-year survival rates of 84%, 50% and 42%.
There is increased PBC prevalence in Thessaly with remarkable geographic clustering and seasonal variability. PBC is diagnosed at early stages although males had a more advanced disease. GLOBE score applies perfectly in Greek patients and this will likely help detecting patients that may benefit from new therapies.
原发性胆汁性胆管炎（PBC）患病率呈上升趋势，且存在显著的地域差异。为描述希腊一大群PBC患者的患病率、空间和时间分布、基线特征、治疗反应、结局以及GLOBE评分的有效性，因为迄今为止尚未对该评分进行独立验证。
回顾性评估和分析过去16年中482例PBC患者（86.5%为女性），使用前瞻性收集的数据库。特别关注根据GLOBE评分评估治疗反应。
初次评估时的年龄为56.3±13.7岁。在432名色萨利居民中，患病率为582/百万（分布不均一）。19个地区的患病率>800/百万。91例患者可确定有症状性疾病发作，春季有一个显著高峰（P=0.03）。诊断时，43.6%无症状，16.2%为肝硬化。男性（P=0.02）、年龄较大（P<0.001）、饮酒（P<0.01）和合并肝脏疾病（P<0.001）是肝硬化的负面预后因素。在中位[四分位间距，范围]随访5.1（7.8，15.7）年期间，62例患者死亡或接受肝移植。GLOBE评分>0.30的患者预后明显更差（P<0.001），5年、10年和15年生存率分别为84%、50%和42%。
色萨利地区PBC患病率增加，具有显著的地理聚集性和季节性变化。PBC在早期被诊断出来，尽管男性患者疾病进展更严重。GLOBE评分在希腊患者中应用良好，这可能有助于发现可能从新疗法中获益的患者。
