Porto-sinusoidal vascular disorder and nephrotic-range proteinuria due to venous vasculitis in Behçet's disease.

Autor: van der Houwen TB; Department of Medicine, Division of Clinical Immunology and Allergy, Vasculitis Center of Expertise, Amsterdam Institute for Immunology and Infectious Diseases, Amsterdam University Medical Center, location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands., Annink ME; Department of Medicine, Division of Clinical Immunology and Allergy, Vasculitis Center of Expertise, Amsterdam Institute for Immunology and Infectious Diseases, Amsterdam University Medical Center, location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands., Roelofs JJTH; Department of Pathology, Amsterdam Cardiovascular Sciences, Amsterdam University Medical Center, location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands., Takkenberg RB; Department of Gastroenterology and Hepatology, Amsterdam University Medical Center, location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands., van Laar JAM; Department of Medicine, Division of Clinical Immunology and Allergy and Department of Immunology, Erasmus University Medical Center, Erasmus University Rotterdam, Rotterdam, the Netherlands., van der Weerd NC; Department of Nephrology, Amsterdam University Medical Center, location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands., Hak AE; Department of Medicine, Division of Clinical Immunology and Allergy, Vasculitis Center of Expertise, Amsterdam Institute for Immunology and Infectious Diseases, Amsterdam University Medical Center, location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands; Department of Rheumatology, Amsterdam University Medical Center, location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands., Kwakernaak AJ; Department of Medicine, Division of Clinical Immunology and Allergy, Vasculitis Center of Expertise, Amsterdam Institute for Immunology and Infectious Diseases, Amsterdam University Medical Center, location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands; Department of Nephrology, Amsterdam University Medical Center, location Academic Medical Center, University of Amsterdam, Amsterdam, the Netherlands. Electronic address: a.j.kwakernaak@amsterdamumc.nl.
Jazyk: angličtina
Zdroj: Clinical immunology (Orlando, Fla.) [Clin Immunol] 2024 Jun; Vol. 263, pp. 110207. Date of Electronic Publication: 2024 Apr 11.
DOI: 10.1016/j.clim.2024.110207
Abstrakt: Behçet's disease (BD) is an autoinflammatory disease with multifactorial and polygenic etiology, potentially involving arteries and veins of any size resulting in variable vessel vasculitis. We report a case of an Iranian male who presented with porto-sinusoidal vascular disorder due to venous vasculitis as initial manifestation of BD. Despite immunosuppression, anticoagulation and venous recanalization, he subsequently developed severe nephrotic-range proteinuria mimicking a primary renal disease which was completely and immediately ameliorated by stenting of the vena cava. This demonstrates that the proteinuria was caused by increased intraglomerular pressure due to venous outflow obstruction as a consequence of venous vasculitis. To our knowledge, this is the first report of massive proteinuria caused by venous obstruction of the caval vein in the context of BD. Altogether, this case demonstrates the extensive spectrum of vascular disease in BD.
(Copyright © 2024 The Authors. Published by Elsevier Inc. All rights reserved.)
Databáze: MEDLINE
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