Mulinacci Giacomo, Palermo Andrea, Cristoferi Laura, Invernizzi Pietro, Carbone Marco
Division of Gastroenterology, Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milan Bicocca, Milan, Italy.
European Reference Network on Hepatological Diseases (ERN RARE-LIVER), San Gerardo Hospital, Monza, Italy.
J Transl Autoimmun. 2021 Sep 21;4:100124. doi: 10.1016/j.jtauto.2021.100124. eCollection 2021.
Takayasu arteritis and primary sclerosing cholangitis are two rare disorders. The pathogenesis of Takayasu arteritis involves immune-mediated mechanisms, and corticosteroids represent the mainstay for treatment. Conversely, the aetiology of primary sclerosing cholangitis remains elusive, even if dysimmunity seems to be one of the contributors to bile duct damage. Despite this, immunosuppressants do not alter disease course. In this paper we describe the association of these two rare disorders, with an unexpected normalization of cholestatic enzymes following steroid treatment. This might hint a novel subtype of sclerosing cholangitis with a prevalent immunebackground, or a local manifestation of Takayasu arteritis.
高安动脉炎和原发性硬化性胆管炎是两种罕见的疾病。高安动脉炎的发病机制涉及免疫介导机制，皮质类固醇是主要的治疗药物。相反，原发性硬化性胆管炎的病因仍然不明，尽管免疫功能异常似乎是胆管损伤的原因之一。尽管如此，免疫抑制剂并不能改变疾病进程。在本文中，我们描述了这两种罕见疾病的关联，以及类固醇治疗后胆汁淤积酶意外恢复正常的情况。这可能暗示了一种以免疫背景为主的新型硬化性胆管炎亚型，或者是高安动脉炎的局部表现。
