Bilateral pulmonary artery aneurysms with protein C and protein S deficiency in a patient with Behçet's disease

Beh?et's disease is a chronic inflammatory disorder of unknown aetiology, characterized by recurrent attacks. Pulmonary artery aneurysm is a rare but serious complication of Beh?et's disease. We describe a patient with Beh?et's disease and protein C and S deficiency who developed bilateral pulmonary artery aneurysms.     

Double coronary thrombosis in a patient with Behçet's disease

Behçet's disease is a chronic relapsing multisystem autoinflammatory condition, in which cardiac involvement is rare, but among the most life-threatening complications. Treatment is largely empirical, and is aimed at suppressing vasculitis. In this role glucocorticoids and colchicine are frequently used.We present the case of a 42-year-old male with previously diagnosed Behçet's disease presenting to our emergency department with an anterior-inferior STEMI. He presented combined thrombosis of the distal anterior descending coronary artery and proximal right coronary artery, and was treated with sequential primary percutaneous coronary interventions and implantation of drug-eluting stents, but required two interventions due to high thrombotic load.His clinical course during hospitalization was good, with no systolic dysfunction at discharge. During follow-up, he has so far had no new cardiovascular events.     

Cardiac thrombosis in a patient with Beh?et's disease: Two years follow-up

A 28-year-old man with Behçet's disease was presented with cardiac symptoms in addition to previous complaints of oral and genital ulcers. A diagnosis of thrombosis was made and patient began to receive anticoagulant and immunosupressive therapy and was followed by echocardiographic examination. Despite medical therapy, thrombosis recurred. Surgical excision was performed and histological findings were consistent with organizing thrombus. Nature of cardiac involvement and review of literature on cardiac thrombosis in Behçet's disease was discussed.     

Left anterior descending coronary artery pseudoaneurysm compressing the main pulmonary artery in a patient with Behçet's disease

Behçet's disease is a systemic inflammatory vascular disorder that may affect the cardiovascular system. Left anterior descending (LAD) artery aneurysm is a rare but potentially fatal complication of Behçet's disease. An accurate diagnosis of this clinical entity requires multimodal imaging (computerized tomography, angiography, and echocardiography) and is essential to guide appropriate therapeutic management. We report the case of a 22‐year‐old female with LAD pseudoaneurysm who underwent surgical repair with transesophageal echocardiography guidance. We describe the importance of the use of an alternative view to define the origin and location of this unusual pathology. (Echocardiography 2012;29:E91‐E93)     

Association of right heart thrombosis,endomyocardial fibrosis,and pulmonary artery aneurysm in Behçet's disease

A 29-year-old man with a 5-year history of Beh?et's disease was admitted for fever, dyspnae, chest pain, and hemoptysis. A diagnosis of right ventricle and atrial thrombosis associated with a pulmonary artery aneurysm was made. The patient was treated with anticoagulants and prednisone. Since hemoptysis persisted, surgical excision of the intracardiac thrombosis was performed and histological findings were consistent with organizing thrombus and endomyocardial fibrosis. Transesophageal echocardiography 6 months later showed recurrence of the right ventricle thrombosis. A course of 6-monthly boluses of intravenous cyclophosphamide was begun. Currently, at 2 years of follow-up, the patient is asymptomatic.     

Aortic arch aneurysm, pseudocoarctation, and coronary artery disease in a patient with Behçet's syndrome

Aortic arch aneurysm, pseudocoarctation, and coronary artery stenosis are extremely rare in Beh?et's syndrome. We present the case of a 25-year-old man with Beh?et's syndrome who underwent coronary artery bypass grafting for severe stenosis in the proximal left anterior descending coronary artery and concomitant surgical correction of a saccular aneurysm that was causing pseudocoarctation of the aortic arch. The surgery was successful.     

A Behçet's disease patient with intracardiac thrombus, pulmonary artery aneurysms complicating recurrent pulmonary thromboembolism

Intracardiac thrombus and pulmonary embolism is a very rare manifestation of Beh?et's disease. A twenty-years-old man was admitted to hospital due to dyspnea, haemoptysis, fever and partially loss of vision. On dynamic thorax computed tomography (CT), there was aneurysmatic dilatation and thrombus in bilateral pulmonary artery segments and also findings of pulmonary thromboembolism. A diagnosis of Beh?et's disease was made based on his clinical course and radiological findings. During treatment, the patient was admitted two times to the hospital because of recurrent pulmonary thromboembolism. At the 10th months of follow up, partially dissolution of the thrombi and pulmonary defects were observed and right ventricular thrombus was revealed by dynamic thorax CT. On a follow up period of 16 months the patient is still under treatment and doing well. We present this case because Beh?et's disease is a rarely considered cause of recurrent pulmonary embolism and intracardiac thrombus which is seen under treatment.     

Achilles tendonitis in a patient with Behçet's disease

Abstract

A 56-year-old male patient had suffered from recurrent oral aphthae, acne-like rashes on the face, an erythema nodosum-like rash on the left lower leg, and severe heel pain on the left-hand side. Colonoscopy revealed six ulcerative lesions in the terminal ileum. Ultrasonography and magnetic resonance imaging showed an inflammatory lesion in the Achilles tendons, with greater inflammation on the left. Achilles tendonitis was considered to be a peripheral enthesopathy in this patient with Behçet's disease complicated by an ileal ulcer.     

Activated protein C levels in Behçet's disease and risk of venous thrombosis

Behçet's disease is a multi‐systemic inflammatory disorder of unknown cause. Most abnormalities have been associated with endothelial injury caused by vasculitis. Thrombosis occurs in about 25% of patients, although the mechanism is unknown. The objective of this study was to evaluate the protein C activation system in Behçet's disease and its correlation with venous thromboembolism (VTE). Thirty‐nine patients (12 with VTE) and 78 age‐ and sex‐matched controls were included in the study, and levels of protein C, protein S, activated protein C (APC), protein C inhibitor (PCI), soluble thrombomodulin (TM), antithrombin (AT), α₁‐antitrypsin, fibrinogen, factor VIII, von Willebrand factor (VWF) and C‐reactive protein (CRP) were measured. APC and TM levels were significantly lower in patients than in controls, whereas protein S, AT, α₁‐antitrypsin, fibrinogen, factor VIII, VWF and CRP levels were significantly higher in patients than in controls. APC, PCI and TM levels were lower in patients with VTE (0·65 ± 0·19 ng/ml, 86% ± 22% and 15·5 ± 7·1 ng/ml respectively) than in those without VTE (0·78 ± 0·17 ng/ml, 100% ± 15% and 22·1 ± 15·3 ng/ml) (P < 0·05). In patients, APC levels below 0·75 ng/ml (10th percentile of the control group) increased the risk of VTE about fivefold (odds ratio = 5·1; 95% confidence interval = 1·1–23·4). These results show that reduced APC levels are associated with the high incidence of VTE in Behçet's disease.     

Achilles tendonitis in a patient with Behçet's disease

A 56-year-old male patient had suffered from recurrent oral aphthae, acne-like rashes on the face, an erythema nodosum-like rash on the left lower leg, and severe heel pain on the left-hand side. Colonoscopy revealed six ulcerative lesions in the terminal ileum. Ultrasonography and magnetic resonance imaging showed an inflammatory lesion in the Achilles tendons, with greater inflammation on the left. Achilles tendonitis was considered to be a peripheral enthesopathy in this patient with Behçet's disease complicated by an ileal ulcer.     

Erosive arthritis in a patient with Behçet's disease

We report on a 35-year-old female patient suffering from Behçet's disease for 20 years who developed recurrent oral ulcerations during the onset of the disease. This was followed by recurrent genital ulcerations and joint symptoms involving knee, wrist and feet joints and bilateral heel pain. X-rays of the feet revealed erosive and destructive changes in the 1st metatarsophalangeal and 1st proximal interphalangeal joints of the right foot and enthesopathy on calcaneus. A literature review indicated that erosive arthritis is a rare event in Behçet's disease.     

Dermographism and atopy in patients with Beh?et's disease.

Dermographism is the appearance of whealing and erythema within minutes in a site where skin has been exposed to pressure or mechanical irritation. In our clinical observations, dermographism seems to be frequent in patients with Beh?et's disease. Since the prevalence of allergic responses is reportedly increased in vasculitic syndromes, we aimed to assess dermographism and atopy in a group of Beh?et's patients. The study group comprised 30 consecutive patients with Beh?et's disease. The study was carried out in two parts: elicited dermographism and atopy tests. In the first part, dermographism was investigated in the study group (Beh?et's patients) and 230 healthy controls. In the second part, the study group, 30 healthy controls and 17 patients with allergic bronchial asthma were evaluated for specific and nonspecific atopy. Dermographism was found to be increased in patients with Beh?et's disease. Peripheral blood eosinophil count, serum total IgE and nasal eosinophil scores were similar in patients with Beh?et's disease and healthy controls. Although blood eosinophil count and serum total IgE levels were higher in allergic bronchial asthma patients than in Beh?et's patients, the difference was significant only in the former. Skin prick test was positive in three of the patients with Beh?et's disease and in six healthy controls. The difference was statistically insignificant (p > 0.05). None of the Beh?et's disease patients had pathergy or a pathergy-like reaction at the site of the skin prick test after 48 h. In conclusion, our results confirm the general impression that dermographism is common in patients suffering from Beh?et's disease.     

Catastrophic antiphospholipid syndrome in a patient with Behçet's disease

We report on a patient who had a life-threatening relapse of Beh?et's disease associated with a catastrophic antiphospholipid syndrome. The patient experienced over a short time a recurrent acute myocardial infarction, multiple venous thromboses, uveitis, and erythema nodosum. Search for thrombophilic factors was positive only for lupus anticoagulant (LAC) and criteria for the diagnosis of the antiphospholipid antibody syndrome were fulfilled. LAC was not found three months after the discharge. At that time the patient had no evidence of clinically active disease or thrombosis. We suggest that LAC was the main triggering factor for the repeated thromboses in this patient.