Digestive Disease Institute, Cleveland Clinic Foundation, Cleveland, Ohio 44195, USA.
Inflamm Bowel Dis. 2010 Sep;16(9):1598-619. doi: 10.1002/ibd.21219.
Diseases involving the hepatopancreatobiliary (HPB) system are frequently encountered in patients with inflammatory bowel disease (IBD). Hepatobiliary manifestations constitute some of the most common extraintestinal manifestations of IBD. They appear to occur with similar frequency in patients with Crohn's disease or ulcerative colitis. HPB manifestations may occur in following settings: 1) disease possibly associated with a shared pathogenetic mechanism with IBD including primary sclerosing cholangitis (PSC), small-duct PSC/pericholangitis and PSC/autoimmune hepatitis overlap, acute and chronic pancreatitis related to IBD; 2) diseases which parallel structural and physiological changes seen with IBD, including cholelithiasis, portal vein thrombosis, and hepatic abscess; and 3) diseases related to adverse effects associated with treatment of IBD, including drug-induced hepatitis, pancreatitis (purine-based agents), or liver cirrhosis (methotrexate), and reactivation of hepatitis B, and biologic agent-associated hepatosplenic lymphoma. Less common HPB manifestations that have been described in association with IBD include autoimmune pancreatitis (AIP), IgG4-associated cholangitis (IAC), primary biliary cirrhosis (PBC), fatty liver, granulomatous hepatitis, and amyloidosis. PSC is the most significant hepatobiliary manifestation associated with IBD and poses substantial challenges in management requiring a multidisciplinary approach. The natural disease course of PSC may progress to cirrhosis and ultimately require liver transplantation in spite of total proctocolectomy with ileal-pouch anal anastomosis. The association between AIP, IAC, and elevated serum IgG4 in patients with PSC is intriguing. The recently reported association between IAC and IBD may open the door to investigate these complex disorders. Further studies are warranted to help understand the pathogenesis of HPB manifestations associated with IBD, which would help clinicians better manage these patients. An interdisciplinary approach, involving gastroenterologists, hepatologists, and, in advanced cases, general, colorectal, and transplant surgeons is advocated.
涉及肝胆系统的疾病在炎症性肠病（IBD）患者中经常遇到。肝胆表现构成了 IBD 最常见的肠外表现之一。它们似乎在克罗恩病或溃疡性结肠炎患者中以相似的频率出现。肝胆表现可能发生在以下情况下：1）与 IBD 可能具有共同发病机制的疾病，包括原发性硬化性胆管炎（PSC）、小胆管PSC/胆管周围炎和 PSC/自身免疫性肝炎重叠、与 IBD 相关的急性和慢性胰腺炎；2）与 IBD 所见的结构和生理变化平行的疾病，包括胆石症、门静脉血栓形成和肝脓肿；3）与 IBD 治疗相关的不良反应相关的疾病，包括药物性肝炎、胰腺炎（嘌呤类药物）或肝硬化（甲氨蝶呤）以及乙型肝炎再激活和生物制剂相关的肝脾淋巴瘤。与 IBD 相关的描述较少见的肝胆表现包括自身免疫性胰腺炎（AIP）、IgG4 相关胆管炎（IAC）、原发性胆汁性肝硬化（PBC）、脂肪肝、肉芽肿性肝炎和淀粉样变性。PSC 是与 IBD 相关的最重要的肝胆表现，在管理方面存在重大挑战，需要多学科方法。PSC 的自然疾病过程可能进展为肝硬化，尽管进行了全直肠结肠切除术和回肠袋肛门吻合术，最终仍需要进行肝移植。AIP、IAC 和 PSC 患者血清 IgG4 升高之间的关联引人注目。最近报道的 IAC 与 IBD 之间的关联可能为研究这些复杂疾病打开了大门。需要进一步的研究来帮助理解与 IBD 相关的肝胆表现的发病机制，这将有助于临床医生更好地管理这些患者。提倡采用涉及胃肠病学家、肝病学家的跨学科方法，在晚期病例中还涉及普外科、结直肠外科和移植外科医生。
