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OBJECTIVES: This study aimed to evaluate the cause of thrombosis in Behçet's disease (BD) patients, since abnormalities in coagulation and fibrinolytic parameters have shown contradictory results. METHODS: Haemostatic parameters were retrospectively evaluated in BD patients treated between January 2007 and January 2011 at Sultan Qaboos University Hospital, Oman. The blood samples of 35 Omani BD patients and 30 healthy controls were analysed for factor VIII:C levels, activated protein C resistance (APCR), von Willebrand factor (vWF) antigens (Ag), collagen binding and ristocetin co-factor activity (RiCoF), antithrombin (AT), protein C (chromogenic and clotting), protein S, homocysteine, tissue plasminogen activator, plasminogen activator inhibitor, plasminogen, alpha 2-antiplasmin, lupus anticoagulant and anticardiolipin and beta2-glycoprotein-1 antibodies. RESULTS: The mean values of factor VIII:C, vWF Ag, AT and protein S were significantly higher in the patient group (P = 0.01, 0.006, 0.04 and 0.01, respectively). There was no deficiency in protein C. Screening for APCR, anticardiolipin antibodies, anti-beta2-glycoprotein-1 antibodies and lupus anticoagulant was negative and there were no differences in homocysteine levels, nor were there differences between patients with and without thrombosis. Six patients had elevated factor VIII:C levels (>150 IU/dL, P <0.02) which normalised on repeat measurements after three months. CONCLUSION: The elevation of factors VIII:C, vWF Ag and AT most likely represent an acute phase phenomenon. In this study, thrombophilic factors did not seem to explain thrombotic tendency. Therefore, further mechanistic studies in a larger group of patients are needed to elucidate the basis for thrombosis in BD. We hypothesise that active BD causes vasculitic endothelial perturbation with dysfunction, leading to the observed increased propensity for thrombosis.
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Pulmonary complications in leptospirosis, though common, are often unrecognized in a non-endemic area. We report here a patient with leptospirosis and severe pulmonary involvement who was treated with meropenem (1 g every 8 hours), moxifloxacin (400 mg once daily), and high doses of corticosteroids. Systemic steroids were continued for 3 months because of persistent pulmonary lesions.
RESUMO
The objective of the study is to study the pattern, frequency and outcome of vascular events in Omani patients with Behcet's disease (BD). Forty BD patients were recruited. Clinical data parameters were recorded, including age at onset, disease duration, current disease activity, and the vascular manifestations of the disease (current and past events). Disease activity was assessed by ESR, CRP and also clinically. For venous and arterial thrombotic events, imaging studies, such as Doppler ultrasonography, angiography, MRI, CT, and echocardiography were performed. Nine patients had vascular events (23%). The mean age of these patients at diagnosis of the vascular event was 24 years (range 15-37) and the mean disease duration at onset of the vascular event was 3.8 years (range 3-5); this was significant when compared with patients without thrombosis (P=0.001, Mann-Whitney test). In these patients with vascular involvement, two patients (22%) had venous lesions, three (33%) patients had arterial lesions and four patients (44%) had mixed lesions. Two patients (22%) had pulmonary artery aneurysm (PAA) and concomitant intracardiac thrombus, and further one patient also had PAA. Immunosuppressive therapy resulted in the resolution of PAA and intracardiac thrombous. In conclusion, the frequency of vascular complications of BD in our patients is similar to those reported around the world. We found younger male patients with short disease duration are at a greater risk of developing thrombosis. Medical treatment with immunosuppressive therapy without anticoagulation seems successful in treating PAA and intracardiac thrombus.