Olivas Pol, Perez-Campuzano Valeria, Orts Lara, Montironi Carla, Magaz Marta, Ruiz Pablo, Shalaby Sarah, Ojeda Asunción, Rosich Pau, Baiges Anna, Turon Fanny, Lens Sabela, García Pagán Juan Carlos, Hernández-Gea Virginia
Liver Unit, Hospital Clínic, Fundació Recerca Clínic Barcelona- Institut d'investigacions Biomèdiques August Pi i Sunyer (IDIBAPS). Departament de Medicina i Ciències de la Salut. Universitat de Barcelona. Centro de Investigación biomédica Red de Enfermedades Hepáticas y Digestivas (CIBEREHD), Barcelona, Spain.
Barcelona Hepatic Hemodynamic Laboratory, Health Care Provider of the European Reference Network on Rare Liver Disorders (ERN RARE-Liver), AGAUR 2021 SGR 01115, Barcelona, Spain.
JHEP Rep. 2023 Dec 27;6(3):100996. doi: 10.1016/j.jhepr.2023.100996. eCollection 2024 Mar.
BACKGROUND & AIMS: Porto-sinusoidal vascular disorder (PSVD) encompasses a group of liver diseases with vascular abnormalities that can cause portal hypertension in the absence of cirrhosis. The new diagnostic criteria allow for coexistence with other liver diseases, however its relationship with chronic hepatitis B (CHB) remains unclear. This study aimed to assess HBV prevalence in a PSVD cohort and evaluate its clinical impact.
This retrospective study was conducted on patients with PSVD at Hospital Clínic Barcelona. HBV serology was evaluated, and patients were categorized into HBV chronic infection, past infection, or no HBV exposure. Clinical characteristics and outcomes were compared.
We included 155 patients with PSVD. Prevalence of CHB and past HBV infection in patients with PSVD was higher than in the general population (5.8% . 0.5%, <0.0001 and 20% 9.1%, <0.0001, respectively). Patients with CHB had a significant delay in PSVD diagnosis compared to those without CHB (11 [5-25] . 1 [0-3] years, = 0.002) and had a more advanced disease (MELD score 12 [9-17] . 9 [7-11], = 0.012) at the time of PSVD diagnosis. The clinical evolution of PSVD in patients with CHB was marked by a significantly higher transplantation rate at the last follow-up (33% 4.1%, = 0.001).
Recognizing the coexistence of PSVD and CHB is important for timely diagnosis and optimal management, highlighting the potential benefits of specialized care for potentially improved outcomes.
The new diagnostic criteria for porto-sinusoidal vascular disorder (PSVD) allow for coexistence with other liver diseases. The results of the present study highlight, for the first time, a non-negligible prevalence of chronic hepatitis B in the PSVD population that was previously unknown. Coexistence may challenge and delay the PSVD diagnosis and is associated with a more unfavorable clinical course. Our findings will increase awareness of this coexistence and improve PSVD diagnosis and management. Furthermore, the data will encourage new studies to determine the prevalence and clinical behavior of other chronic liver diseases that coexist with PSVD.
门静脉-肝窦血管紊乱(PSVD)包括一组伴有血管异常的肝脏疾病,这些疾病在无肝硬化的情况下可导致门静脉高压。新的诊断标准允许其与其他肝脏疾病共存,然而其与慢性乙型肝炎(CHB)的关系仍不明确。本研究旨在评估PSVD队列中HBV的流行情况并评估其临床影响。
本回顾性研究在巴塞罗那临床医院对PSVD患者进行。评估HBV血清学,将患者分为HBV慢性感染、既往感染或无HBV暴露。比较临床特征和结局。
我们纳入了155例PSVD患者。PSVD患者中CHB和既往HBV感染的患病率高于一般人群(分别为5.8%对0.5%,P<0.0001和20%对9.1%,P<0.0001)。与无CHB的患者相比,CHB患者的PSVD诊断明显延迟(11[5-25]年对1[0-3]年,P=0.002),且在PSVD诊断时疾病进展更严重(终末期肝病模型评分12[9-17]对9[7-11],P=0.012)。在最后一次随访时,CHB患者中PSVD的临床进展以显著更高的移植率为特征(33%对4.1%,P=0.001)。
认识到PSVD和CHB的共存对于及时诊断和优化管理很重要,突出了专科护理对潜在改善结局的潜在益处。
门静脉-肝窦血管紊乱(PSVD)的新诊断标准允许其与其他肝脏疾病共存。本研究结果首次突出了PSVD人群中慢性乙型肝炎的患病率不可忽视,而这一情况此前并不为人所知。共存可能会挑战并延迟PSVD的诊断,且与更不利的临床病程相关。我们的研究结果将提高对这种共存情况的认识,并改善PSVD的诊断和管理。此外,这些数据将鼓励开展新的研究,以确定与PSVD共存的其他慢性肝病的患病率和临床行为。