Mertz Andrew, Nguyen Nhu An, Katsanos Konstantinos H, Kwok Ryan M
Department of Internal Medicine (Andrew Mertz), Walter Reed National Military Medical Center Bethesda, MD, USA.
Gastroenterology (Nhu An Nguyen, Ryan M. Kwok), Walter Reed National Military Medical Center Bethesda, MD, USA.
Ann Gastroenterol. 2019 Mar-Apr;32(2):124-133. doi: 10.20524/aog.2019.0344. Epub 2019 Jan 15.
Comorbid primary sclerosing cholangitis (PSC) and inflammatory bowel disease (IBD) represent a unique disease phenotype with a different risk profile than PSC or IBD alone. While the pathogenetic mechanisms behind both diseases remain unclear, recent studies have targeted several immune-mediated pathways in an attempt to find a potential therapeutic target. Patients with PSC-associated IBD typically exhibit pancolitis with a right-to-left intestinal inflammatory gradient associated with a greater incidence of backwash ileitis and rectal sparing. Although there is an increased incidence of pancolitis in this population, bowel symptoms tend to be less significant than in IBD alone. Likewise, the degree of inflammation and symptoms of PSC-associated IBD are characteristically less clinically significant. Despite the relatively quiescent clinical presentation of PSC-associated IBD, there is an increased risk for colorectal and hepatobiliary malignancy making vigilance for malignancy essential.
原发性硬化性胆管炎（PSC）与炎症性肠病（IBD）并存代表了一种独特的疾病表型，其风险特征与单独的PSC或IBD不同。虽然这两种疾病背后的发病机制仍不清楚，但最近的研究针对了几种免疫介导的途径，试图找到潜在的治疗靶点。PSC相关IBD患者通常表现为全结肠炎，伴有从右到左的肠道炎症梯度，伴有更高的反流性回肠炎发病率和直肠 spared 。虽然该人群中全结肠炎的发病率有所增加，但肠道症状往往不如单独的IBD明显。同样，PSC相关IBD的炎症程度和症状在临床上通常不太显著。尽管PSC相关IBD的临床表现相对静止，但结直肠癌和肝胆恶性肿瘤的风险增加，因此对恶性肿瘤保持警惕至关重要。 （注：原文中“rectal sparing”不太明确其准确含义，推测可能是“直肠 spared”，这里保留原文未翻译准确的部分）
