LIVER TRANSPLANTATION IN PATIENTS WITH PRIMARY SCLEROSING CHOLANGITIS: A MULTICENTRIC STUDY.

Henrique de Aguiar Wiederkehr, Julio Cesar Wiederkehr, Mauro Rafael Da Igreja, Eduardo Brommelstroet Ramos, Marcelo Scheidemantel Nogara, Debora Stroparo Soffiatti, Andrew Massutti, Vivian Laís Sasaki, Barbara de Aguiar Wiederkehr, Igor Raphael Mathias Valejo, Júlio Cezar Uili Coelho
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Abstract

Background: The prevalence of primary sclerosing cholangitis (PSC) in the general population has not yet been clearly established. The management of PSC should focus on delaying the progression of the disease and restraining its complications. The only curative therapy for the disease remains liver transplantation (LT). PSC is currently the fifth most common indication for LT and corresponds to 5% of all LT indications in adults.

Aims: Our objective is to evaluate the indications and outcomes of PSC patients undergoing LT in three liver transplantation centers in southern Brazil - Hospital Santa Isabel in Blumenau, Santa Catarina state, and Hospital das Clínicas and Hospital Nossa Senhora das Graças, in Curitiba, Parana state).

Methods: This is a longitudinal observational study of patients with PSC who underwent LT in three major Brazilian medical centers. Electronic medical records and study protocols of all patients subjected to LT from January 2011 to December 2021 were retrospectively reviewed.

Results: Of the 1,362 transplants performed in the three medical centers, 37 were due to PSC. Recurrence of PSC occurred in three patients (8.1%) in 3.0±2.4 years (range, 1-4 years). The 1-year and 5-year survival rates after the first LT were 83.8 and 80.6%, respectively. The 1-year and 5-year graft survival rates were, respectively, 83.8 and 74.8%.

Conclusions: Our experience with LT in patients with PSC demonstrated good patient and graft survival results. Most deaths were due to common factors in patients undergoing LT.

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原发性硬化性胆管炎患者的肝移植:一项多中心研究。
背景:原发性硬化性胆管炎(PSC)在普通人群中的患病率尚未明确确定。PSC的管理应侧重于延缓疾病进展和抑制其并发症。这种疾病的唯一治疗方法仍然是肝移植。PSC是目前LT第五常见的适应症,相当于成人所有LT适应症的5%。目的:我们的目标是评估在巴西南部三个肝移植中心接受LT的PSC患者的适应症和结果,这三个中心分别是位于圣卡塔琳娜州布鲁门瑙的Santa Isabel医院和位于库里蒂巴的Clínicas医院和Nossa Senhora das Graças医院,方法:这是一项对在巴西三个主要医疗中心接受LT的PSC患者的纵向观察研究。对2011年1月至2021年12月接受LT的所有患者的电子病历和研究方案进行了回顾性审查。结果:在三个医疗中心进行的1362例移植中,37例是PSC。PSC在3.0±2.4年(范围1-4年)内复发3例(8.1%)。首次LT后的1年和5年生存率分别为83.8%和80.6%。1年和5年移植物存活率分别为83.8%和74.8%。结论:我们对PSC患者进行LT的经验表明,患者和移植物的存活率良好。大多数死亡是由LT患者的常见因素引起的。
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