General Condition:
Among the 12 diagnosed children with VBS, there were 5 males and 7 females. Among them, 10 cases were treated at the Rheumatology and Immunology Department of the Capital Institute of Pediatrics, accounting for 21.2% of the total number of BS children during the same period. The total count stood at 45, including 15 males and 30 females. The 10 cases included 4 males and 7 females. The occurrence rate of VBS in male children with Behçet's syndrome was 23.5%, while in female children, it was 20%. There was no significant statistical difference between the two groups. The median age of onset of the 12 VBS cases was 9.5 years (range: 3–13 years). The median time from onset to detection of vascular lesions was 8 months (range: 3 weeks to 2 years and 6 months). Vascular lesions were found concurrently with diagnosis in 10 cases (10/12), and 4 months before diagnosis in one case (1/12). In one case (1/12), vascular lesions were detected 2 years and 6 months after onset during disease activity.
Clinical Manifestations
Among the 12 cases, 11 (11/12) presented with oral ulceration (Table 1). Skin manifestations were observed in 10 cases (10/12), including skin nodules or erythema nodosum in 5 cases, positive skin prick test in 3 cases, and folliculitis in 3 cases. Fever was reported in 9 cases (9/12), while gastrointestinal involvement was seen in 9 cases (9/12), including abdominal pain, diarrhoea, and haematochezia reported in 6 cases (6/12), gastrointestinal ulceration in 6 cases (6/12), and thickening of the gastrointestinal wall in 4 cases. Genital ulceration was observed in 6 cases (6/12), and perianal ulceration was noted in 4 cases (4/12). In addition, urological manifestations were present in 6 cases (6/12), including proteinuria in 4 cases, sterile pyuria in 1 case, and microscopic haematuria in 1 case. Hypertension was recorded in 5 cases (5/12), including asymmetrical blood pressure in 3 cases (3/12) and weakened pulse in 1 case (1/12). Neurological manifestations were observed in 5 cases (5/12), including hypertensive encephalopathy in 1 case, cerebral venous sinus thrombosis in 2 cases, Adams–Stokes syndrome in 1 case, and cerebral infarction due to thrombus detachment in 1 case. Joint pain or arthritis was reported in 3 cases (3/12), while ocular involvement was seen in 3 cases (3/12). Finally, one case (1/12) had heart murmur.
Vascular Manifestations
Among the 12 cases, arterial involvement was observed in 10 cases (10/12) (Table 2), with 4 cases (4/12) showing venous involvement. Among those with vascular involvement, 8 cases (8/12) had isolated arterial involvement, 2 cases (2/12) had both arterial and venous involvement, and 2 cases (2/12) had isolated venous involvement. Vascular lesions included arterial wall thickening, luminal stenosis, arterial occlusion, arterial aneurysm formation, and intravascular thrombosis.
In terms of arterial involvement, among the vascular lesions, arterial wall thickening was observed in 6 cases (6/12), luminal stenosis in 5 cases (5/12), pulmonary artery thrombosis in 2 cases (2/12), and pulmonary artery aneurysm in 1 case (1/12) (Fig. 1). Arterial occlusion was identified in 2 cases (2/12), affecting the anterior tibial artery and dorsalis pedis artery in one case each. Among the affected sites, 3 cases (3/12) involved the pulmonary artery, including 2 cases of pulmonary artery thrombosis and 1 case of pulmonary artery aneurysm. Four cases (4/12) had involvement of the main arteries, including 1 case of ascending aortic aneurysm (1/12), 1 case of thickening of the aortic arch with luminal stenosis (1/12), and 2 cases (2/12) with involvement of both thoracic and abdominal aortas, with 2 cases of wall thickening and 1 case of luminal stenosis. Among the branch vessels, there were 2 cases (2/12) involving the brachiocephalic trunk, with 2 cases of wall thickening and 1 case of luminal stenosis. Two cases (2/12) showed wall thickening with luminal stenosis of the carotid artery, while the subclavian artery and superior mesenteric artery were affected in 3 cases each (3/12), with 3 cases of wall thickening and 2 cases of luminal stenosis. One case (1/12) involved the celiac trunk with wall thickening and luminal stenosis. Three cases (3/12) involved the renal artery, all with single renal artery involvement, with 1 case showing wall thickening with luminal stenosis and 1 case of luminal stenosis. Coronary artery dilation was observed in 2 cases (2/12). One case (1/12) involving the upper limb artery showed wall thickening with luminal stenosis in the axillary artery. Among the lower limb arteries, there were 4 cases (4/12), including 2 cases of superficial femoral artery involvement, 2 cases involving the femoral artery and anterior tibial artery, and 1 case each of involvement of the popliteal artery, posterior tibial artery, and dorsalis pedis artery. Among the 4 cases of venous involvement, 2 cases (2/12) showed venous wall thickening with luminal stenosis, and 2 cases (2/12) presented with venous thrombosis. One case (1/12) witnessed superficial venous thrombosis (SVT) in the upper and lower limbs, and one case (1/12) demonstrated thrombosis of the intracranial venous sinuses (Fig. 2), accompanied by thrombosis of the inferior vena cava, renal veins, and internal jugular vein.
Laboratory Investigations (Table 3): Hematologic parameters revealed a median white blood cell count of 16.91 (range 6.54–27.25) × 109/L, elevated in 8 out of 11 cases. Median haemoglobin was 104 (range 68–133) g/L, with anaemia present in 7 cases out of 10. Platelet count median was 412.5 (range 310–737) × 109/L, elevated in 8 out of 10 cases. The median C-reactive protein level was 79.5 (range 2.6–159) mg/L, elevated in 11 out of 12 cases. Median erythrocyte sedimentation rate was 76 (range 5-129) mm/1h, elevated in 11 out of 12 cases. Serum ferritin median was 165 (range 25.39–512) ng/ml, elevated in 5 out of 11 cases. Humoral immunity showed a median serum immunoglobulin G of 14.35 (range 8.61–25.1) g/L, elevated in 6 out of 10 cases, and serum immunoglobulin A median of 2.38 (range 1.67–5.15) g/L, with 6 cases elevated out of 10. Three cases out of 12 were positive for direct antiglobulin test, 2 cases out of 12 for antinuclear antibodies and anti-cardiolipin antibodies, and 1 case out of 12 for lupus anticoagulant or anti-Ro52 antibodies. Tests for EB virus nucleic acid and tuberculosis infection T-cell spot were negative in all 12 cases.
Treatment and Prognosis (Table 4): All 12 cases received steroid therapy, 11 cases out of 12 were treated using steroids combined with immunosuppressive agents, 9 cases out of 12 were treated using steroids combined with biologic agents, and 8 cases out of 12 were treated using steroids combined with thalidomide. Immunosuppressive therapy included the use of cyclophosphamide in 9 cases out of 12, with 3 cases out of 12 switching to azathioprine after discontinuation of cyclophosphamide, and 3 cases out of 12 maintained on tofacitinib. Two cases out of 12 received methotrexate during the course, and 5 cases out of 12 received mycophenolate mofetil. Among biologic agents, all 9 cases received tumour necrosis factor antagonists, with 3 cases out of 12 receiving infliximab alone, 4 cases out of 12 receiving adalimumab alone, 2 cases transitioning from infliximab to adalimumab, and 1 case switching to tocilizumab after ineffectiveness of tumour necrosis factor antagonists. Four cases out of 12 underwent surgical treatment, including 1 case of incision and drainage for skin rash, 1 case of resection of a large arterial aneurysm in the left lower lung, 1 case of aortic valve repair, and 1 case of Bentall procedure combined with pulmonary artery thrombus removal and mechanical aortic valve fixation.
Prognosis and Complications (Table 5): The median follow-up duration was 2 years (range 4 months to 5 years). One case was lost to follow-up, one case died (sudden cardiac death), 8 cases remained stable, and 2 cases experienced recurrent activity. Complications included steroid-induced cataracts in 2 cases and cerebral infarction after thrombus detachment in 1 case.