Pruritus, Fatigue, Osteoporosis and Dyslipoproteinemia in Pbc Patients: A Clinician’s Perspective

S. Dražilová, Tomas Koky, Marian Macej, M. Janíčko, D. Simkova, Ariunzaya Tsedendamba, Slavomira Komarova, Peter Jarcuska
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Abstract

In this review article, we summarize the most common clinical manifestations of Primary biliary cholangitis (PBC): pruritus, fatigue, osteoporosis, and dyslipoproteinemia and discuss their impact of the patients’ quality of life. More than half of PBC patients suffer from pruritus or fatigue at the time of diagnosis. We discuss the pathophysiological aspects of the PBC clinical manifestations and treatment options. The pathophysiology of pruritus and fatigue is not adequately elucidated, but IL-31 is associated with the severity of pruritus and could be used to objectify the subjective reporting by questionnaires. Although PBC patients suffer from atherogenic dyslipidemia, they do not seem to have a higher cardiovascular risk; however, this observation needs to be clarified by further clinical studies. The second-line of PBC treatment affects pruritus severity: Obeticholic acid (OCA) worsens pruritus while fibrates improve it. Itching can be alleviated by both non-pharmacological and pharmacological approach, however the are multiple barriers to pharmacological treatment. There is no adequate treatment for fatigue today. Treatment of osteoporosis and dyslipidemia is similar for non-PBC patients; stage of liver disease should be considered in treatment. Further research to clarify the pathophysiology and to eventually discover an effective treatment to improve survival and quality of life (especially pruritus and fatigue) in PBC patients is needed.
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肺结核患者的瘙痒、疲劳、骨质疏松症和脂蛋白异常血症:临床医生的视角
在这篇综述文章中,我们总结了原发性胆汁性胆管炎(PBC)最常见的临床表现:瘙痒、疲劳、骨质疏松症和脂蛋白异常血症,并讨论了它们对患者生活质量的影响。半数以上的 PBC 患者在确诊时患有瘙痒症或疲劳症。我们将讨论 PBC 临床表现的病理生理学方面和治疗方案。瘙痒和疲劳的病理生理学尚未充分阐明,但IL-31与瘙痒的严重程度有关,可用于客观化问卷调查的主观报告。虽然 PBC 患者患有致动脉粥样硬化性血脂异常,但他们似乎并没有更高的心血管风险;不过,这一观察结果还需要进一步的临床研究来澄清。PBC 的二线治疗会影响瘙痒的严重程度:奥贝胆酸(OCA)会加重瘙痒,而纤维素类药物则会改善瘙痒。瘙痒可通过非药物和药物方法缓解,但药物治疗存在多种障碍。目前还没有治疗疲劳的适当方法。骨质疏松症和血脂异常的治疗方法与非良性前列腺增生症患者相似;治疗时应考虑肝病的分期。我们需要进一步开展研究,以阐明病理生理学,并最终找到一种有效的治疗方法来改善 PBC 患者的生存和生活质量(尤其是瘙痒和疲劳)。
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