Diffuse generalized venulitis as the primary pathology of Behçet's disease: A comprehensive magnetic resonance venography study

Abstract

Objective: Behçet's Disease (BD) is a chronic multisystem vasculitis that manifests with destructive inflammation affecting the eyes, central nervous system, and blood vessels. The pathology of vein involvement in BD is poorly characterized. Magnetic resonance (MR) venography gives more comprehensive information about deep veins and adjacent tissues. In this study, we aimed to characterize vein involvement and evaluate the diagnostic utility of MR venography in BD.

Methods: Sixty-five BD patients who fulfilled the International Study Group (ISG) criteria and 20 healthy control subjects were enrolled. Inferior vena cava (IVC), common iliac veins (CIV), external (EIV) and internal iliac veins (IVV), common femoral veins (CFV), femoral veins (FV), and greater saphenous veins (GSV) of BD patients and healthy controls were evaluated with MR venography and ultrasonography for the presence pathologic features, luminal thrombi, vessel wall changes, and perivascular abnormalities.

Results: 33 vascular and 32 non-vascular BD patients (mean age 39.3 ± 11.3 years and 48 [73.8%] male) were enrolled. MR venography revealed diffuse concentric thickening of the walls of IVC, CIV, EIV, IIV, CFV, FV, and GSV in BD (healthy controls vs. BD p<0.05 for all vein segments). MR venography provided additional information about veins and perivascular tissues like contrast enhancement, enlarged lymph nodes, and seminal vesicle vascularization, which were remarkably more frequent in vascular BD than non-vascular BD and healthy controls.

Conclusion: The results of our study suggest that the involvement of the venous system is diffuse and generalized in BD, and demonstration of venulitis might help diagnose the disease.

Keywords: Behçet's disease; Common femoral veins; Common iliac veins; Diagnose; Inferior vena cava; Magnetic resonance; Thrombosis; Vascular involvement; Vein; Venography.