Search

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. • Porto-sinusoidal vascular disorder, due to venous vasculitis, can be a primary manifestation of BD • This report is the first to describe proteinuria resulting from thrombosis due of the inferiorvena cava in BD • Proteinuria in absence of renal vein thrombosis should raise suspicion of a vascular problemdistal from the renal vasculature [ABSTRACT FROM AUTHOR]