Porto-sinusoidal vascular disorder in chronic HBV: A significant coexistence not to be overlooked
Pol Olivas,
Valeria Perez-Campuzano,
Lara Orts,
Carla Montironi,
Marta Magaz,
Pablo Ruiz,
Sarah Shalaby,
Asunción Ojeda,
Pau Rosich,
Anna Baiges,
Fanny Turon,
Sabela Lens,
Juan Carlos García Pagán,
Virginia Hernández-Gea
Affiliations
Pol Olivas
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
Valeria Perez-Campuzano
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
Lara Orts
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
Carla Montironi
Department of Pathology, Hospital Clínic de Barcelona, Barcelona, Spain
Marta Magaz
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
Pablo Ruiz
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
Sarah Shalaby
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
Asunción Ojeda
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
Pau Rosich
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
Anna Baiges
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
Fanny Turon
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
Sabela Lens
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
Juan Carlos García Pagán
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
Virginia Hernández-Gea
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; Corresponding author. Address: Barcelona Hepatic Hemodynamic Laboratory, Liver Unit, Hospital Clínic, Villarroel 170, Barcelona 08036, Spain. Tel.: +34932275400 (2209), fax: +34 932279856.
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. Methods: 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. Results: 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% vs. 0.5%, p <0.0001 and 20% vs. 9.1%, p <0.0001, respectively). Patients with CHB had a significant delay in PSVD diagnosis compared to those without CHB (11 [5–25] vs. 1 [0–3] years, p = 0.002) and had a more advanced disease (MELD score 12 [9–17] vs. 9 [7–11], p = 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% vs. 4.1%, p = 0.001). Conclusions: 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. Impact and implications: 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.