Héla Kchir, Asma Ouaka Kchaou, Ali Amouri, Nadia Ben Mahmoud, Mohamed Moussadek Azzouz, Nabil Tahri, Abdeljabbar Ghorbel, Béchir Zouari, Nabyl Ben Mami
Tunis Med. 2014 May;92(5):323-8.
Primary sclerosing cholangitis (PSC) is a chronic cholestatic disease strongly associated, in the western series, to inflammatory bowel disease (IBD) and particularly to chronic ulcerative colitis. North African data are rare. aim : To study the epidemiological, clinicobiological and morphological profile of PSC in Tunisia. methods: A retrospective multicenter study extended over a period of 14 years (1995-2009), including all patients suffering from PSC, hospitalized in the four participants departments. We collected epidemiological, clinico-biological, histopathologic and morphological data for each patient. results: We brought together 33 patients (22 men and 11 women), middle aged 44 years. The disease was symptomatic in 73% of cases. Laboratory tests showed cholestasis (100%) associated with hyperbilirubinemia (72%) and a moderate cytolysis (78%). Morphological analysis of bile ducts by cholangioMRI or endoscopic retrograde cholangio-pancreatography objectified diffuse damage of the biliary tract in 61% of cases. Association with IBD was found in 33% of cases (Crohn's Disease: 27%, chronic ulcerative colitis: 6%). An association with autoimmune hepatitis was found in 6% of cases and primary biliary cirrhosis in 3% of cases. Conclusion : PSC is rare in Tunisia and affects men more often than women. The association with IBD is less frequent than in literature. It concerns essentially Crohn's disease. These data require confirmation by prospective multicenter studies.
原发性硬化性胆管炎（PSC）是一种慢性胆汁淤积性疾病，在西方病例系列中与炎症性肠病（IBD）密切相关，尤其是与慢性溃疡性结肠炎相关。北非的数据很少。目的：研究突尼斯原发性硬化性胆管炎的流行病学、临床生物学和形态学特征。方法：一项回顾性多中心研究，时间跨度为14年（1995 - 2009年），纳入所有在四个参与研究的科室住院的原发性硬化性胆管炎患者。我们收集了每位患者的流行病学、临床生物学、组织病理学和形态学数据。结果：共纳入33例患者（22例男性和11例女性），平均年龄44岁。73%的病例有症状。实验室检查显示胆汁淤积（100%）伴有高胆红素血症（72%）和中度细胞溶解（78%）。通过磁共振胰胆管造影（MRCP）或内镜逆行胰胆管造影（ERCP）对胆管进行形态学分析发现，61%的病例存在胆道弥漫性损伤。33%的病例发现与炎症性肠病有关（克罗恩病：27%，慢性溃疡性结肠炎：6%）。6%的病例发现与自身免疫性肝炎有关，3%的病例与原发性胆汁性肝硬化有关。结论：原发性硬化性胆管炎在突尼斯很少见，男性比女性更易受累。与炎症性肠病的关联比文献报道的频率低。主要涉及克罗恩病。这些数据需要前瞻性多中心研究予以证实。
