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Background

Behçet’s disease (BD) is a variable vessel vasculitis and vascular involvement is one of its life threatening manifestations. Arterial involvement frequently occurs with male predominance with pseudoaneurysms being the most common presentation. Immunosuppressive therapy is the mainstay of treatment in vascular involvement.

Case presentation

The case we report here is a 40 year old Iraqi BD patient with manifestations of recurrent oral and genital ulcers, bilateral anterior uveitis, and deep vein thrombosis. The pathergy test was positive. The HLA-B51 was negative, erythrocyte sedimentation rate 102 mm/1st h and C-reactive protein was 48 mg/L. After discontinuation of his medications for about 9 months, the disease presented with leg pain and swelling that was diagnosed as huge left superficial femoral artery pseudoaneurysm by Doppler ultrasonography. CT angiography revealed a 90 × 88 × 70 mm pseudoaneurysm with partial mural thrombosis. He was scheduled for emergency surgery due to severe intractable pain. he received a pulse of methylprednisolone 1 g/day for 3 days and then surgery was done in the form of exclusion, repair and femorofemoral bypass were done. Post-operatively, the patient had an uneventful course; distal pulses became palpable, pain and swelling subsided. Post-operation, prednisolone 1 mg/kg was continued and he received cyclophosphamide 750 mg intravenously. His blood homocysteine level was higher than normal 23.8 μmol/L. He was discharged with a high dose of steroid and monthly cyclophosphamide treatment.

Conclusion

Arterial pseudoaneurysm is life-threatening in BD and should be kept in mind to prevent major complications. Vascular involvement in BD patients is probably associated with hyperhomocysteinemia.  相似文献   
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BackgroundSystemic sclerosis (SSc) is a connective tissue disease affecting the skin and multiple organs. Most of the evidence on ocular involvement comes from small studies. We evaluated the retinal vascular changes in patients with SSc and its associations with types, disease duration, skin score, and nailfold capillaroscopy changes.Material and methodsWe evaluated the demographic, clinical and nailfold capillaroscopy data of 52 patients referring to the clinic of scleroderma with SSc according to the 2013 ACR/EULAR SSc criteria. In addition, full ophthalmic examination in 52 patients and fundus photography in 40 patients were done in the ophthalmology clinic.ResultsThere were 52 (49 women and 3 men, 17 diffuse and 35 limited) patients with SSc with mean disease duration of 8 ±5 years and mean age of 40 ±9 years. Retinal changes in the ophthalmologic examination were seen in 30.7% as increased vascular tortuosity. None of the patients had cotton wool spot, hemorrhage or hard exudate. Forty patients underwent fundus photography and 22.5% of them had vascular tortuosity. Only the presence of hemorrhage in the nailfolds was correlated with retinal tortuosity, and the other characteristics of nailfold capillaroscopy did not have any association with retinal changes.ConclusionsRetinal vascular changes were seen in about one third of our patients. There was no correlation between nailfold capillaroscopy, disease duration, type and skin score of SSc patients and retinal vascular changes. These findings suggest that the mechanisms or the quality of changes in the retinal vessels and nailfold vasculature may be different.  相似文献   
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