Autoimmune biomarkers in porto‐sinusoidal vascular disease: Potential role in its diagnosis and pathophysiology

Background and aims Porto‐sinusoidal vascular disease (PSVD) is a rare disease that requires excluding cirrhosis and other causes of portal hypertension for its diagnosis because it lacks a specific diagnostical test. Although it has been occasionally associated with autoimmune diseases, the pathoph...

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Bibliographic Details
Published in:Liver international 2021-09, Vol.41 (9), p.2171-2178
Main Authors: Cerda Reyes, Eira, González‐Navarro, Europa Azucena, Magaz, Marta, Muñoz‐Sánchez, Guillermo, Diaz, Alba, Silva‐Junior, Gilberto, Triguero, Ana, Lafoz, Erica, Campreciós, Genís, Orts, Lara, Perez‐Campuzano, Valeria, Seijo, Susana, Rubio, Laura, Turon, Fanny, Baiges, Anna, Hernández‐Gea, Virginia, Álvarez‐Larran, Alberto, Juan, Manel, Garcia‐Pagan, Juan Carlos
Format: Article
Language:English
Subjects:
AECA
Antibodies
Autoantibodies
Autoimmune diseases
Autoimmunity
Biomarkers
Cirrhosis
Diagnosis
Disease
Endothelial cells
Enzyme-linked immunosorbent assay
Hematological diseases
Hepatocytes
Hypertension
idiopathic non‐cirrhotic portal hypertension
Immunofluorescence
INCPH
Liver
Liver cirrhosis
Pathophysiology
porto‐sinusoidal vascular disease
PSVD immunological biomarkers
Sine waves
Splenomegaly
Statistical methods
Vascular diseases
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Summary:Background and aims Porto‐sinusoidal vascular disease (PSVD) is a rare disease that requires excluding cirrhosis and other causes of portal hypertension for its diagnosis because it lacks a specific diagnostical test. Although it has been occasionally associated with autoimmune diseases, the pathophysiology of PSVD remains unknown. The aim of this study was to evaluate the potential role of autoimmunity in the pathophysiology and diagnosis of PSVD. Methods Thirty‐seven consecutive patients with PSVD and 39 with cirrhosis matched by gender, signs of portal hypertension and liver function were included (training set). By using Indirect Immunofluorescence, ELISA and slot‐blot methods data 22 autoantibodies were identified in patients with PSVD and cirrhosis. Presence of anti‐endothelial cells antibodies (AECA) was assayed by a cell‐based ELISA. Thirty‐one PSVD, 40 cirrhosis patients, 15 patients with splenomegaly associated with haematological disease and 14 healthy donors were included in a validation set. Findings The proportion of patients with at least one positive antibody was statistically significantly higher in patients with PSVD compared with cirrhosis (92% vs 56%; P 
ISSN:1478-3223
1478-3231
DOI:10.1111/liv.14997