Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease often associated with inflammatory bowel disease (IBD), particularly ulcerative colitis (CU), and rarely with Crohn’s disease (CD). Various long-term analyses show different rates of cancer and the need for orthotopic liver transplantation (OLT) in patients with isolated PSC and with concomitant IBD, respectively. However, data on the detailed course of PSC with or without IBD are limited. We aimed to analyze the clinical disease course of PSC patients without IBD compared to PSC patients with UC and CD, respectively. A retrospective data analysis of patients with isolated PSC (n = 41) and of patients with concomitant IBD (n = 115) was performed. In detail, PSC disease characteristics including occurrence of dominant stenoses, liver cirrhosis, OLT and malignancy, as well as the temporal course of PSC activity and disease progression, were analyzed. A multivariable Cox regression model and a Fine–Gray competing risk model were further used for the independent risk factor analysis of cirrhosis development and OLT. Patients with isolated PSC were significantly older at first diagnosis than patients with PSC-IBD (39 vs. 28 years, p = 0.02). A detailed analysis of the course of PSC revealed a faster PSC progression after initial diagnosis in isolated PSC patients compared to PSC-IBD including significantly earlier diagnosis of dominant stenoses (29 vs. 74 months, p = 0.021) and faster progression to liver cirrhosis (38 vs. 103 months, p = 0.027). Patients with isolated PSC have a higher risk of developing cirrhosis than patients with PSC-IBD (Gray’s test p = 0.03). OLT was more frequently performed in male patients with isolated PSC compared to males with coincident IBD (48% (n = 13) vs. 33% (n = 25), p = 0.003). Colorectal carcinoma was significantly more often diagnosed in patients with PSC-IBD than in isolated PSC (8.7% vs. 0%, p = 0.042). Patients with isolated PSC seem to have a different clinical course of disease than PSC patients with concomitant IBD characterized by a more pro-fibrotic disease course with earlier onset of liver cirrhosis and dominant stenosis but with less malignancy. These data may be interpreted as either a more progressive disease course of isolated PSC or a later diagnosis of the disease at an advanced disease stage. The different clinical courses of PSC and the underlying mechanisms of the gut–liver axis need further attention.

中文翻译：

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孤立的 PSC 患者肝纤维化进展加剧以及 PSC-IBD 队列中恶性肿瘤风险增加：一项回顾性研究

原发性硬化性胆管炎 (PSC) 是一种慢性胆汁淤积性肝病，通常与炎症性肠病 (IBD)，特别是溃疡性结肠炎 (CU) 相关，很少与克罗恩病 (CD) 相关。各种长期分析显示，孤立性 PSC 患者和伴有 IBD 的患者的癌症发生率不同，并且需要进行原位肝移植 (OLT)。然而，有关伴有或不伴有 IBD 的 PSC 详细病程的数据有限。我们的目的是分别分析不伴有 IBD 的 PSC 患者与患有 UC 和 CD 的 PSC 患者的临床病程。对孤立性 PSC 患者 (n = 41) 和伴有 IBD 的患者 (n = 115) 进行了回顾性数据分析。详细分析了 PSC 疾病特征，包括显性狭窄、肝硬化、OLT 和恶性肿瘤的发生，以及 PSC 活动和疾病进展的时间过程。多变量Cox回归模型和Fine-Gray竞争风险模型进一步用于肝硬化发展和OLT的独立危险因素分析。首次诊断时，孤立性 PSC 患者的年龄显着高于 PSC-IBD 患者（39 岁 vs. 28 岁，p = 0.02）。对 PSC 病程的详细分析显示，与 PSC-IBD 相比，孤立的 PSC 患者在初次诊断后 PSC 进展更快，包括显着早期诊断显性狭窄（29 个月与 74 个月，p = 0.021）和更快进展为肝硬化（ 38 个月与 103 个月，p = 0.027）。孤立性 PSC 患者比 PSC-IBD 患者发生肝硬化的风险更高（格雷检验 p = 0.03）。与同时患有 IBD 的男性患者相比，患有孤立性 PSC 的男性患者更常进行 OLT（48% (n = 13) vs. 33% (n = 25)，p = 0.003）。PSC-IBD 患者中结直肠癌的诊断率明显高于单纯 PSC 患者（8.7% vs. 0%，p = 0.042）。孤立性 PSC 患者似乎与伴有 IBD 的 PSC 患者具有不同的临床病程，其特征是更促纤维化的病程，肝硬化和显性狭窄发病较早，但恶性程度较低。这些数据可以解释为分离的 PSC 的更进展的疾病过程或晚期疾病阶段的疾病的后期诊断。PSC的不同临床过程和肠肝轴的潜在机制需要进一步关注。