Guifarro Daniel A, De Oliveira-Gomes Diana, Beas Renato, Yibirin-Wakim Marcel J, Montalvan-Sanchez Eleazar E
Department of Medicine, National Autonomous University of Honduras, Tegucigalpa, HND.
Department of Medicine, University of Texas Southwestern Medical Center, Dallas, USA.
Cureus. 2023 Mar 14;15(3):e36126. doi: 10.7759/cureus.36126. eCollection 2023 Mar.
Overlap syndrome (OS) is a term that comprises the presentation of multiple hepatic disease characteristics in the same patient, such as the presence of autoimmune hepatitis (AIH) features in addition to primary sclerosing cholangitis (PSC) or primary biliary cholangitis (PBC). Standard therapy for AIH is immunosuppression, while ursodeoxycholic acid is the preferred treatment for PBC. Additionally, liver transplantation (LT) may be considered in severe cases. Hispanics have been found to have a higher prevalence of chronic liver disease and develop more complications associated with portal hypertension at the time of listing for LT. Despite being the fastest-growing population in the USA, Hispanics have a higher probability of not receiving an LT due to issues with social determinants of health (SDOH). It has been reported that Hispanics are more likely to be removed from the transplant list. We report a case of a 25-year-old female immigrant from a Latin American developing country who presented with symptoms consistent with worsening liver disease after years of inappropriate workup and late diagnosis due to barriers within the healthcare system. The patient had a history of unresolved jaundice and pruritus and presented with worsening of her previous symptoms and new onset abdominal distention, bilateral leg edema, and telangiectasias. Laboratory and imaging studies confirmed the diagnosis of AIH and primary sclerosing cholangitis (PSC-AIH syndrome). The patient was started on steroids, azathioprine, and ursodeoxycholic acid, showing improvement. Due to her migratory status, she could not receive an appropriate diagnosis and follow up with a single provider or healthcare institution, putting her at increased risk for life-threatening complications. Although medical management is the first step, the probability of future liver transplants exists. The patient is still undergoing liver transplant evaluation and completing a workup since she was found to have an elevated model for end-stage liver disease (MELD) score. Even with the introduction of new scores and policies that aim to reduce disparities in LT, Hispanic patients are still at higher risk of being removed from the waitlist because of death or clinical deterioration compared to non-Hispanics. To this day, Hispanics have the highest percentage of waitlist deaths (20.8%) of all ethnicities and the lowest overall rate for undergoing LT. Understanding and addressing the causes that could contribute to and explain this phenomenon is essential. Increasing awareness of this problem is vital to promote more research on LT disparities.
重叠综合征(OS)是一个术语,指同一患者出现多种肝脏疾病特征,例如除原发性硬化性胆管炎(PSC)或原发性胆汁性胆管炎(PBC)外还存在自身免疫性肝炎(AIH)特征。AIH的标准治疗是免疫抑制,而熊去氧胆酸是PBC的首选治疗药物。此外,严重病例可考虑肝移植(LT)。已发现西班牙裔慢性肝病患病率较高,在列入肝移植名单时出现更多与门静脉高压相关的并发症。尽管西班牙裔是美国增长最快的人群,但由于健康的社会决定因素(SDOH)问题,他们接受肝移植的可能性较低。据报道,西班牙裔更有可能被从移植名单中除名。我们报告一例来自拉丁美洲发展中国家的25岁女性移民病例,该患者在多年因医疗系统内的障碍导致检查不当和诊断延迟后,出现了与肝病恶化相符的症状。患者有未解决的黄疸和瘙痒病史,此次出现先前症状加重以及新发腹胀、双侧下肢水肿和毛细血管扩张。实验室和影像学检查确诊为AIH和原发性硬化性胆管炎(PSC-AIH综合征)。患者开始使用类固醇、硫唑嘌呤和熊去氧胆酸治疗,病情有所改善。由于她的移民身份,她无法由单一医疗服务提供者或医疗机构进行适当诊断和随访,这使她面临危及生命并发症的风险增加。尽管药物治疗是第一步,但未来仍有肝移植的可能性。该患者仍在接受肝移植评估并完成相关检查,因为她的终末期肝病模型(MELD)评分升高。即使引入了旨在减少肝移植差异的新评分和政策,与非西班牙裔相比,西班牙裔患者因死亡或临床恶化而被从等待名单中除名的风险仍然更高。时至今日,西班牙裔在所有种族中等待名单死亡率最高(20.8%),接受肝移植的总体比例最低。了解并解决可能导致和解释这一现象的原因至关重要。提高对这一问题的认识对于促进更多关于肝移植差异的研究至关重要。