PDF(Pubmed)
Abstract：
UNASSIGNED: Autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC) can co-exist in AIH-PBC, requiring combined treatment with immunosuppression and ursodeoxycholic acid (UDCA). The Paris criteria are commonly used to identify these patients; however, the optimal diagnostic criteria are unknown. We aimed to evaluate the use and clinical relevance of both Paris and Zhang criteria.
UNASSIGNED: Eighty-three patients with a clinical suspicion of AIH-PBC who were treated with combination therapy were included. Histology was re-evaluated. Characteristics and long-term outcomes were retrospectively compared to patients with AIH and PBC.
UNASSIGNED: Seventeen (24%) patients treated with combination therapy fulfilled the Paris criteria. Fifty-two patients (70%) fulfilled the Zhang criteria. Patients who met Paris and Zhang criteria more often had inflammation and fibrosis on histology compared to patients only meeting the Zhang criteria. Ten-year liver transplant (LT)-free survival was 87.3% (95% CI 78.9-95.7%) in patients with AIH-PBC. This did not differ in patients in or outside the Paris or Zhang criteria (p = 0.46 and p = 0.40, respectively) or from AIH (p = 0.086). LT-free survival was significantly lower in patients with PBC and severe hepatic inflammation - not receiving immunosuppression - compared to those with AIH-PBC (65%; 95% CI 52.2-77.8% vs. 87%; 95% CI 83.2-90.8%; hazard ratio 0.52; p = 0.043).
UNASSIGNED: In this study, patients with AIH-PBC outside Paris or Zhang criteria were frequently labeled as having AIH-PBC and were successfully treated with combination therapy with similar outcomes. LT-free survival was worse in patients with PBC and hepatic inflammation than in those treated as having AIH-PBC. More patients may benefit from combination therapy.
UNASSIGNED: This study demonstrated that patients with AIH-PBC variant syndrome treated with combined therapy consisting of immunosuppressants and ursodeoxycholic acid often do not fulfill the Paris criteria. They do however have comparable response to therapy and long-term outcomes as patients who do fulfill the diagnostic criteria. Additionally, patients with PBC and additional signs of hepatic inflammation have poorer long-term outcomes compared to patients treated as having AIH-PBC. These results implicate that a larger group of patients with features of both AIH and PBC may benefit from combined treatment. With our results, we call for improved consensus among experts in the field on the diagnosis and management of AIH-PBC variant syndrome.
UNASSIGNED: Eighty-three patients with a clinical suspicion of AIH-PBC who were treated with combination therapy were included. Histology was re-evaluated. Characteristics and long-term outcomes were retrospectively compared to patients with AIH and PBC.
UNASSIGNED: Seventeen (24%) patients treated with combination therapy fulfilled the Paris criteria. Fifty-two patients (70%) fulfilled the Zhang criteria. Patients who met Paris and Zhang criteria more often had inflammation and fibrosis on histology compared to patients only meeting the Zhang criteria. Ten-year liver transplant (LT)-free survival was 87.3% (95% CI 78.9-95.7%) in patients with AIH-PBC. This did not differ in patients in or outside the Paris or Zhang criteria (p = 0.46 and p = 0.40, respectively) or from AIH (p = 0.086). LT-free survival was significantly lower in patients with PBC and severe hepatic inflammation - not receiving immunosuppression - compared to those with AIH-PBC (65%; 95% CI 52.2-77.8% vs. 87%; 95% CI 83.2-90.8%; hazard ratio 0.52; p = 0.043).
UNASSIGNED: In this study, patients with AIH-PBC outside Paris or Zhang criteria were frequently labeled as having AIH-PBC and were successfully treated with combination therapy with similar outcomes. LT-free survival was worse in patients with PBC and hepatic inflammation than in those treated as having AIH-PBC. More patients may benefit from combination therapy.
UNASSIGNED: This study demonstrated that patients with AIH-PBC variant syndrome treated with combined therapy consisting of immunosuppressants and ursodeoxycholic acid often do not fulfill the Paris criteria. They do however have comparable response to therapy and long-term outcomes as patients who do fulfill the diagnostic criteria. Additionally, patients with PBC and additional signs of hepatic inflammation have poorer long-term outcomes compared to patients treated as having AIH-PBC. These results implicate that a larger group of patients with features of both AIH and PBC may benefit from combined treatment. With our results, we call for improved consensus among experts in the field on the diagnosis and management of AIH-PBC variant syndrome.
摘要：
■自身免疫性肝炎（AIH）和原发性胆汁性胆管炎（PBC）可以在AIH-PBC中共存，需要免疫抑制和熊去氧胆酸（UDCA）联合治疗。巴黎标准通常用于识别这些患者；然而，最佳诊断标准未知.我们旨在评估Paris和Zhang标准的使用和临床相关性。
■83例临床怀疑AIH-PBC的患者接受联合治疗。重新评估组织学。回顾性比较了AIH和PBC患者的特征和长期结局。
■17例（24%）接受联合治疗的患者符合巴黎标准。52例患者（70％）符合Zhang标准。与仅符合Zhang标准的患者相比，符合Paris和Zhang标准的患者在组织学上更经常发生炎症和纤维化。AIH-PBC患者的十年无肝移植（LT）生存率为87.3％（95％CI78.9-95.7％）。在Paris或Zhang标准之内或之外（分别为p=0.46和p=0.40）或AIH（p=0.086）的患者中，这没有差异。与AIH-PBC患者相比，未接受免疫抑制的PBC和严重肝脏炎症患者的无LT生存率显着降低（65％;95％CI52.2-77.8％vs.87%；95%CI83.2-90.8%；风险比0.52；p=0.043）。
■在这项研究中，不符合Paris或Zhang标准的AIH-PBC患者常被标记为AIH-PBC患者,并成功接受联合治疗,结局相似.PBC和肝脏炎症患者的无LT生存率比AIH-PBC患者差。更多的患者可能受益于联合治疗。
■这项研究表明，使用免疫抑制剂和熊去氧胆酸联合治疗的AIH-PBC变异综合征患者通常不符合巴黎标准。然而，与符合诊断标准的患者相比，他们对治疗和长期结局的反应相当。此外,与接受AIH-PBC治疗的患者相比,患有PBC和其他肝脏炎症体征的患者的长期结局较差.这些结果表明，具有AIH和PBC特征的更多患者可能受益于联合治疗。根据我们的结果,我们呼吁该领域的专家就AIH-PBC变异综合征的诊断和治疗达成更多共识.
■83例临床怀疑AIH-PBC的患者接受联合治疗。重新评估组织学。回顾性比较了AIH和PBC患者的特征和长期结局。
■17例（24%）接受联合治疗的患者符合巴黎标准。52例患者（70％）符合Zhang标准。与仅符合Zhang标准的患者相比，符合Paris和Zhang标准的患者在组织学上更经常发生炎症和纤维化。AIH-PBC患者的十年无肝移植（LT）生存率为87.3％（95％CI78.9-95.7％）。在Paris或Zhang标准之内或之外（分别为p=0.46和p=0.40）或AIH（p=0.086）的患者中，这没有差异。与AIH-PBC患者相比，未接受免疫抑制的PBC和严重肝脏炎症患者的无LT生存率显着降低（65％;95％CI52.2-77.8％vs.87%；95%CI83.2-90.8%；风险比0.52；p=0.043）。
■在这项研究中，不符合Paris或Zhang标准的AIH-PBC患者常被标记为AIH-PBC患者,并成功接受联合治疗,结局相似.PBC和肝脏炎症患者的无LT生存率比AIH-PBC患者差。更多的患者可能受益于联合治疗。
■这项研究表明，使用免疫抑制剂和熊去氧胆酸联合治疗的AIH-PBC变异综合征患者通常不符合巴黎标准。然而，与符合诊断标准的患者相比，他们对治疗和长期结局的反应相当。此外,与接受AIH-PBC治疗的患者相比,患有PBC和其他肝脏炎症体征的患者的长期结局较差.这些结果表明，具有AIH和PBC特征的更多患者可能受益于联合治疗。根据我们的结果,我们呼吁该领域的专家就AIH-PBC变异综合征的诊断和治疗达成更多共识.
