Pineau B C, Pattee L P, McGuire S, Sekar A, Scully L J
Department of Medicine, University of Saskatchewan, Saskatoon.
Can J Gastroenterol. 1997 Jan-Feb;11(1):45-8. doi: 10.1155/1997/948191.
A 23-year-old man presenting with acute pancreatitis and autoimmune hemolytic anemia was diagnosed with primary sclerosing cholangitis (PSC) without evidence of ulcerative colitis. This constellation of rare associations constitutes a unique mode of presentation of PSC. Within two years he also developed ankylosing spondylitis with sacroiliitis. Disordered immune regulation as a major factor in the mechanism of injury in PSC is supported by its increased association with other immunologically mediated disorders, most notably ulcerative colitis. Autoimmune hemolytic anemia, however, has been reported to be associated with PSC on only two occasions, and ankylosing spondylitis in the absence of ulcerative colitis is also unusual. In addition, the presentation of PSC with acute pancreatitis has rarely been described. This patient presented with several unusual features of PSC.
一名23岁男性，表现为急性胰腺炎和自身免疫性溶血性贫血，被诊断为原发性硬化性胆管炎（PSC），且无溃疡性结肠炎证据。这种罕见关联的组合构成了PSC的一种独特表现形式。两年内他还患上了伴有骶髂关节炎的强直性脊柱炎。PSC损伤机制中的主要因素是免疫调节紊乱，这一点得到了它与其他免疫介导疾病（最显著的是溃疡性结肠炎）关联增加的支持。然而，据报道自身免疫性溶血性贫血仅在两例中与PSC相关，且在无溃疡性结肠炎的情况下出现强直性脊柱炎也不常见。此外，很少有关于PSC伴急性胰腺炎的报道。该患者呈现出PSC的几个不寻常特征。
