Johnson Adejoke M, Akpan Ezekiel J, Kale Supriya, Patel Anna
Medicine, All Saints University School of Medicine, Roseau, DMA.
Medicine, All Saints University College of Medicine, Kingstown, VCT.
Cureus. 2021 Jul 2;13(7):e16109. doi: 10.7759/cureus.16109. eCollection 2021 Jul.
Primary biliary cholangitis (PBC) is characterized as an autoimmune disease that involves the destruction of intrahepatic bile ducts, characteristically leading to a cholestatic liver. The presence of disease-specific antimitochondrial antibodies (AMA) is the gold standard to diagnose PBC. Typically, PBC is known to affect female populations exceedingly over their male counterparts. Associated autoimmune diseases include Sjogren's and Raynaud's syndrome, which are also more prevalent in women. The low incidence rates of men affected with PBC, especially with a concomitant type 1 diabetes diagnosis, have resulted in little being known about the clinical course of the disease in this particular population group. Current research suggests no significant histological, serological, or biochemical differences between PBC in males and females. However, some symptoms and clinical associations may be different. This case report presents the rare case of a male patient with type 1 diabetes recently diagnosed with PBC.
原发性胆汁性胆管炎(PBC)的特征是一种自身免疫性疾病,涉及肝内胆管的破坏,典型地导致胆汁淤积性肝病。疾病特异性抗线粒体抗体(AMA)的存在是诊断PBC的金标准。通常,已知PBC在女性人群中的影响远远超过男性。相关的自身免疫性疾病包括干燥综合征和雷诺综合征,这些疾病在女性中也更普遍。男性患PBC的发病率较低,尤其是同时诊断为1型糖尿病的男性,导致对这一特定人群中该疾病的临床病程了解甚少。目前的研究表明,男性和女性的PBC在组织学、血清学或生化方面没有显著差异。然而,一些症状和临床关联可能有所不同。本病例报告介绍了一名最近被诊断为PBC的1型糖尿病男性患者的罕见病例。