Division of Gastroenterology and Hepatology, University of Maryland School of Medicine, 22 S. Greene St N3W50, Baltimore, MD, 21201, USA.
Department of Medicine, Tufts University School of Medicine, Washington St, Boston, MA, 02111, USA.
Hepatol Int. 2023 Jun;17(3):720-734. doi: 10.1007/s12072-022-10468-8. Epub 2022 Dec 27.
Patients with autoimmune hepatitis (AIH) may co-present with features of primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC). Using a national transplant registry, the outcomes of patients with these autoimmune liver conditions were compared.
The UNOS-STAR registry was used to select a study population of AIH, PSC, and PBC liver transplant (LT) patients. Living and multi-organ transplant cases were excluded. Using the UNOS-registered diagnoses, the study population was subdivided into those with nonoverlapping autoimmune liver diseases and those with overlapping forms (e.g., AIH-PBC). Outcomes were compared, using endpoints such as all-cause mortality, graft failure, and organ-system specific causes of death.
The main analysis featured 2048 entries, with 1927 entries having nonoverlapping AIH, 52 entries having PSC overlap, and 69 entries having PBC overlap. Patients with PBC overlap were more likely to have graft failure (adjusted hazard ratio [aHR] 3.46 95% CI 1.70-7.05), mortality secondary to respiratory causes (aHR 3.57 95% CI 1.23-10.43), and mortality secondary to recurrent disease (aHR 9.53 95% CI 1.85-49.09). Case incidence rates reflected these findings, expressed in events per 1000 person-years. For patients with PBC overlap and nonoverlapping AIH cases, respectively. Graft failure: 28.87 events vs. 9.42 events, mortality secondary to respiratory causes: 12.83 deaths vs. 3.77 deaths, mortality secondary to recurrent disease: 6.42 deaths vs. 1.26 deaths. Those with AIH-PSC overlap experienced a higher risk of death from graft infection (aHR 10.43 95% CI 1.08-100.37; case-incidence rate: 3.89 vs. 0.31 mortalities per 1000 person-years). Supplementary analysis showed similar findings, in which overlapping autoimmune conditions were associated with higher adverse outcome rates.
Patients with AIH-PBC overlap have higher risk of mortality due to recurrent liver disease and respiratory causes, and patients with AIH-PSC overlap have higher risk of mortality due to graft infection. While further prospective studies are needed to clarify the underlying mechanisms related to these findings, our study characterizes the prognostic implications of AIH overlap on post-LT mortality and graft failure risks.
自身免疫性肝炎（AIH）患者可能同时具有原发性胆汁性胆管炎（PBC）或原发性硬化性胆管炎（PSC）的特征。利用国家移植登记处，比较了这些自身免疫性肝病患者的结局。
使用 UNOS-STAR 登记处选择 AIH、PSC 和 PBC 肝移植 (LT) 患者的研究人群。排除活体和多器官移植病例。使用 UNOS 登记的诊断，将研究人群细分为无重叠自身免疫性肝病和重叠形式（例如 AIH-PBC）的患者。使用全因死亡率、移植物失败和器官系统特异性死亡等终点比较结果。
主要分析包括 2048 项，其中 1927 项为非重叠 AIH，52 项为 PSC 重叠，69 项为 PBC 重叠。PBC 重叠患者更易发生移植物失败（调整后的危险比[aHR]3.46，95%CI 1.70-7.05）、呼吸原因导致的死亡率（aHR 3.57，95%CI 1.23-10.43）和复发性疾病导致的死亡率（aHR 9.53，95%CI 1.85-49.09）。发病率以每 1000 人年发生的事件数表示，反映了这些发现。对于 PBC 重叠和非重叠 AIH 病例的患者，分别为：移植物失败：28.87 例与 9.42 例；呼吸原因导致的死亡率：12.83 例与 3.77 例；复发性疾病导致的死亡率：6.42 例与 1.26 例。AIH-PSC 重叠患者因移植物感染而死亡的风险更高（aHR 10.43，95%CI 10.08-100.37；发病率：3.89 例与 0.31 例/1000 人年）。补充分析显示了类似的结果，重叠自身免疫性疾病与更高的不良结局风险相关。
AIH-PBC 重叠患者因复发性肝病和呼吸原因导致的死亡率较高，AIH-PSC 重叠患者因移植物感染导致的死亡率较高。虽然需要进一步的前瞻性研究来阐明与这些发现相关的潜在机制，但本研究描述了 AIH 重叠对 LT 后死亡率和移植物失败风险的预后意义。
