Behçet’s Disease, Associated Large Vessel Thrombosis, and Coexistent Thrombophilia: A Distinct Nosological Entity?

Table 5

Synopsis of the three patients’ diagnostic assessment.

	Cardiovascular thromboses	Thrombophilic factors	HLA	ISG criteria; 1990	Japanese criteria; 2003
Patient A; 32-year old	(i) SVC; (ii) Subclavian vein (right); (iii) Intracardiac (right atrium)	(i) Homozygous MTHFR C677T	B*51; Positive	(1) Recurrent oral aphthosis; +; (2) Genital ulceration; +; (3) Positive pathergy; =; Behçet’s Disease	(1) Verifiable recurrent oral aphthosis; +; (2) Genital ulceration; +; (3) Vascular lesions; =; Possible BD; (i) Positive pathergy; (ii) Vasculitic lesions in SVC and brachiocephalic vein biopsy
Patient B; 54-year old	(i) IVC; (ii) Iliac veins bilaterally; (iii) Femoral veins bilaterally; (iv) Deep veins of lower limbs	(i) Heterozygous MTHFR C677T; (ii) Hyperhomocysteinemia; (iii) Heterozygous FV Leiden R506Q	B*51; Positive	(1) Recurrent oral aphthosis; +; (2) Positive pathergy; =; Insufficient criteria for diagnosis of BD	(1) Verifiable recurrent oral aphthosis; +; (2) Deteriorating vascular lesions; =; Possible BD; (i) Positive pathergy
Patient C; 20-year old	(i) IVC; (ii) Iliac veins bilaterally; (iii) Femoral veins bilaterally; (iv) Spermatic vein; (v) Deep vein of lower limbs	(i) Heterozygous MTHFR C677T; (ii) Hyperhomocysteinemia; (iii) Heterozygous FV Leiden R506Q; (iv) Transitory positive Lupus Anticoagulant	B*51; B*27; Positive	Lacking criteria for diagnosis of BD	(1) Reported episodes of painless oral aphthosis, not observed by any physician; +; (2) Deteriorating vascular lesions; =; Lacking criteria for diagnosis of BD; (i) Negative pathergy; (ii) Normal pathologic findings in small saphenous vein biopsy