Background Behcet's disease is a kind of systemic vasculitis. Multiple organs including derma, mucosae, articulation, neurologic system, gastrointestinal tract and cardiovascular system might be involved. Vital organ complications lead to poor outcomes. Cardiovascular involvement in Behcet's disease is estimated to range from 7% to 40%, with fatality rate reaches up to 29% [1–3].The characteristics of Chinese Behcet's disease patients with cardiovascular lesions were rarely reported before.
Objectives To investigate the clinical characteristics of cardiovascular involvement in Behcet's disease.
Methods We reviewed 35 medical records of patients of Behcet's disease with cardiovascular lesions in Anzhen Hospital, Capital Medical University between January 2002 and January 2014. All the patients fulfilled the international Behcet's disease study group criteria .
Results (1) The mean (±SD) age when the cardiovascular disorders flare-up was 36.9±9.6 years. 27/35 (77.1%) of the patients were male.
(2) The clinical spectrum of cardiovascular abnormalities included valvular regurgitation (20,57.1%, among these patients, two patients complicated with infectious endocarditis, and five patients had perivalvular leakage after primary valvular surgery), conduction system disturbances (10,28.6%), intracardiac thrombosis (1,2.9%), aneurysms of the arteries (13,37.1%), artery stenosis or occlusion (5,14.3%), pulmonary vascular diseases (3,8.6%), venous thrombosis (4,11.4%). 20 patients had more than one of these lesions simultaneously.
(3) Clinical manifestations were varied from shortness of breath (17,48.6%), febrile (9,25.7%), chest pain (5,14.3%), dizziness (4,11.4%), cough (4,11.4%), limb ischemia (4,11.4%), palpitation (3,8.6%), hemoptysis (3,8.6%), seroperitoneum (2,5.7%), facial edema (1,2.9%), dysphagia (1,2.9%), to asymmetric blood pressure (1,2.9%).
(4) Inflammatory indicators erythrocyte sedimentation rate and C-reactive protein were not significantly different between the different subgroups (P=0.26 and P=0.972, respectively).
(5) Ten patients received corticosteroids and immunosuppressive drugs treatment. Intervention therapy (aortic coated stent lumen isolation) was administered on one patient. Operations (including valve replacement, valular annulus reconstruction, Bentall, aorta replacement and heart transplantation) were performed on 12 patients. One patient (2.9%) died from multiple organ dysfunction during hospital stay.
Conclusions Cardiovascular involvement in Behcet's disease is a serious systemic lesion deserved attention. Corticosteroids and immunosuppressive drugs together with intervention therapy or operations are effective remedies for Behcet's disease patients with cardiovascular lesions.
Kajiya T, Anan R, Kameko M, et al. Intracardiac thrombus, superior vena esva syndrome, and pulmonary embolism in a patient with Behcet's disease: a case report and literature review[J]. Heart Vessels, 2007, 22(4):278–283.
Geri G, Wechsler B, Thi Huong du L, et al. Spectrum of cardiac Iesions in Behcet disease: a series of 52 patients and review of the literature[J]. Medicine(Bahimore), 2012, 91(1):25–34.
Desbois A C, Wechsler B, Cluzel P, et al. Cardiovascular involvement in Behcet's disease[J]. Rev Med Interne, 2014, 35(2):103–111.
International study group for Behcet's disease. Lancet, 1990, 335(8697):1078–1080.
Disclosure of Interest None declared