Behcet disease (BD) is a chronic multisystem disease that presents with recurrent oral and genital ulceration and recurrent uveitis. Vascular and cardiac thromboses are rare but life threatening complications. We report a case of right internal jugular vein and brachiocephalic vein thrombus and successive cardiac thrombosis, which was finally diagnosed with BD.
In November 2007, a 29-year-old man presented with swelling of face and cervical part 5 days after a knock on his right neck was hospitalized. Ultrasound, CT and magnetic resonance angiography (MRA) of cervical blood vessels showed multiple thromboses in his right internal jugular vein and brachiocephalic vein (Figure 1A). Erythrocyte sedimentation rate (ESR) varied from 18 mm to 39 mm in the first hour, C-reactive protein (CRP) was 23.9 mg/L (normal range: 0-8 mg/L). Other examinations including echocardiography, ultrasound of other blood vessels, haemogram, hemagglutination, D-dimeride, full set of biochemistry, immune indexes such as antinuclear antibody, immunoglobulin, complement, anticardiolipin antibody, lupus anticoagulant were normal. He was diagnosed as phlebothrombosis and treated with heparin and warfarin by turns. In August 2008, the patient returned with low-grade fever that began 1 month earlier.
On examination, his body temperature, heart rate, blood pressure were 37.7°C, 84 beats/min, 100/60 mmHg, respectively. The right side of his neck was a little more raised than the left side and the superficial vein on the right side was dilated. The skin examination showed multiple 0.5-cm sized folliculitis on both his face and back. No abnormal signs were found in the heart, lungs, abdomen and nerve system.
The ESR was 61 mm in the first hour, CRP was 63.6 mg/L. Echocardiography showed multiple vegetations in mitral valve, aortic valve and endocardium of the left ventricle (Figure 2A). Ultrasound and MRA showed recanalization of right internal jugular vein and formation of bypass circuit of brachiocephalic vein (Figure 1B). Tuberculin skin test with purified protein derivative was positive (+++). The results of chest radiography, sputum culture, hémoculture, ocular examination and other remaining routine laboratory investigations were normal.
During his stay in hospital the patient developed painful ulceration on his margo lateralis linguae mucosa and erythema nodosum on his left leg. These symptoms proved later to be of a recurrent nature: the former began 5 years earlier at the frequency of at least once a month; the latter initiated 1 year earlier on both legs appearing the feature of transmigration. Almost every time at the site of puncture for haemospasia an erythematous nodus with a pustule developed.
The diagnosis of BD was therefore established. Prednisones, methotrexate, thalidomide, wafarrin and anti-tuberculosis drug were used. Gradual improvements of symptoms and results of examination showed one month later the fever disappeared, so did the vegetations of anterior leaflet of mitral valve. ESR and CRP declined to normal ranges. Six months later, the patient returned to hospital. Echocardiography showed all the vegetations disappeared (Figure 2B). Conditions of impaired blood vessels improved on MRA showing extent of diseased diminished, dilation of anodic blood vessel lessened, compensatory circulation formed.
According to the criteria reported by the International Study Group for BD,1 the diagnosis in our case was based upon the presence of recurrent oral ulceration, folliculitis, erythema nodosum, and the positive reaction to venepuncture, the same meaning as the reaction to needling, which is a unique feature of this malady.
BD is recognized as a multisystem disorder, etiology of which is unclear and assumed to be related to many factors such as inheritance, immunology and infections. The main pathological feature of BD is vasculitis which may cause lesions in different tissues. Because of various clinical manifestations and having no specific laboratory examinations, diagnosis of BD is difficult. In our case, the recurrent oral ulcerations and skin lesions were unspecific and failed to absorb patient's and doctor's attention. Besides this, the low incidence of BD and the rare complication of cardiovascular system were also the reason of misdiagnosis.
Vascular thrombosis and cardiac vegetations may be caused by deposition of immune complexes and platelet aggregation on endothelial cells.2,3 Due to high velocity dynamic blood flow within the cardiac chambers, the endocardium is less prone to be damaged from circulating immune complexes, except when the immune system is extensively activated. Cardiac vegetations accompanied with fever, high level of ESR and CRP in this patient reflected the disease was in highly active period.
Immunosuppressive therapy is crucial for BD, especially with cardiovascular complications. Only anticoagulant therapy or resection of thrombus could not prevent the formation of new injuries.4 In our case, considering tuberculosis may be one of etiological factors,5 the possibility of sub-clinical infection and subsequent diffusion of tuberculosis after immunosuppressive therapy, we also used the anti-tuberculosis drugs. Therapeutic efficacy was apparent but the follow-up visit is indispensable for the following reasons: (1) cardiovascular manifestation is a symbol of prognosis mala because mortality of this kind was 20%;6 (2) owing to the relapsing characteristic of BD, new thrombosis may develop even during immunosuppressive treatment.7
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