Eaton John E, McCauley Bryan M, Atkinson Elizabeth J, Juran Brian D, Schlicht Erik M, de Andrade Mariza, Lazaridis Konstantinos N
Division of Gastroenterology and Hepatology, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.
Division of Biomedical Statistics and Informatics, Mayo Clinic College of Medicine and Sciences, Rochester, Minnesota, USA.
J Gastroenterol Hepatol. 2017 Oct;32(10):1763-1768. doi: 10.1111/jgh.13774.
Primary sclerosing cholangitis (PSC) typically develops in middle-age adults. Little is known about phenotypic differences when PSC is diagnosed at various ages. Therefore, we sought to compare the clinical characteristics of a large PSC cohort based on the age when PSC was diagnosed.
We performed a multicenter retrospective review to compare the features of PSC among those diagnosed between 1-19 (n = 95), 20-59 (n = 662), and 60-79 years (n = 102).
Those with an early diagnosis (ED) of PSC were more likely to have small-duct PSC (13%) than those with a middle-age diagnosis (MD) (5%) and late diagnosis (LD) groups (2%), P < 0.01, and appeared to have a decrease risk of hepatobiliary malignancies: ED versus MD: hazard ratio (HR), 0.25; 95% confidence interval (CI) 0.06-1.03, and ED versus LD: HR, 0.07; 95% CI 0.01-0.62. Cholangiocarcinoma was diagnosed in 78 subjects (ED n = 0, MD n = 66, and LD n = 12) and was more likely to be diagnosed within a year after the PSC diagnosis among those found to have PSC late in life: ED 0% (0/95), MD 2% (14/662), and LD 6% (6/102), P = 0.02. Similarly, hepatic decompensation was more common among those with LD-PSC versus younger individuals: LD versus MD: HR, 1.64; 95% CI 0.98-2.70, and LD versus ED: HR, 2.26; 95% CI 1.02-5.05.
Those diagnosed with PSC early in life are more likely to have small-duct PSC and less likely to have disease-related complications. Clinicians should be vigilant for underlying cholangiocarcinoma among those with PSC diagnosed late in life.
原发性硬化性胆管炎（PSC）通常在中年成人中发病。对于在不同年龄诊断出的PSC的表型差异知之甚少。因此，我们试图根据PSC的诊断年龄比较一个大型PSC队列的临床特征。
我们进行了一项多中心回顾性研究，以比较在1 - 19岁（n = 95）、20 - 59岁（n = 662）和60 - 79岁（n = 102）之间诊断出的PSC患者的特征。
PSC早期诊断（ED）组比中年诊断（MD）组（5%）和晚期诊断（LD）组（2%）更易出现小胆管PSC（13%），P < 0.01，且似乎患肝胆恶性肿瘤的风险降低：ED组与MD组相比：风险比（HR）为0.25；95%置信区间（CI）为0.06 - 1.03，ED组与LD组相比：HR为0.07；95% CI为0.01 - 0.62。78例患者被诊断为胆管癌（ED组0例，MD组66例，LD组12例），在晚年被诊断为PSC的患者中，胆管癌更可能在PSC诊断后一年内被诊断出来：ED组0%（0/95），MD组2%（14/662），LD组6%（6/102），P = 0.02。同样，LD - PSC患者比年轻患者更易出现肝失代偿：LD组与MD组相比：HR为1.64；95% CI为0.98 - 2.70，LD组与ED组相比：HR为2.26；95% CI为1.02 - 5.05。
早年被诊断为PSC的患者更易出现小胆管PSC，且发生疾病相关并发症的可能性较小。临床医生应对晚年被诊断为PSC的患者潜在的胆管癌保持警惕。
