Behçet’s Syndrome and Thrombosis

Behçet syndrome (BS) is a multisystem vasculitis with unknown etiology and a unique geographic distribution. The disease course is characterized by exacerbations and remissions while abating as the years pass. The usual onset is in the third decade. Recurrent skin mucosa lesions and sight threatening panuveitis are the hallmark of the disease. Males are more severely affected than females. Vascular involvement can occur in up to 40% of cases. BS is unique among the vasculitides in that it may involve all sizes and types of vessels. It affects the veins more than the arteries. Lower extremity vein thrombosis is the most frequent manifestation of vascular involvement, followed by vena cava thrombosis, pulmonary artery aneurysms, Budd-Chiari syndrome, peripheral artery aneurysms, dural sinus thrombosis and abdominal aorta aneurysms. Vascular involvement is frequently associated with constitut onal symptoms and increased acute phase response and is the major cause of increased mortality. A predominantly neutrophilic vasculitis around the vaso vasorum is typical of BS. The thrombus is tightly adherent to the vessel wall which probably explains why thromboembolism is so rare despite the high frequency of venous disease. Thrombophilic factors do not seem to explain thrombotic tendency in BS. Immunosuppressive treatment is essential in suppression and preventing the attacks

The efficacy of anakinra in an adolescent with colchicine-resistant familial Mediterranean fever

Colchicine is the treatment of choice in familial Mediterranean fever (FMF) for the prevention of both attacks and secondary amyloidosis. The overall nonresponder rate is about 5–10%. Anakinra is known to have good effectiveness in a severe autoinflammatory syndrome [chronic infantile neurological cutaneous and articular (CINCA) syndrome] and other recurrent hereditary periodic fevers. Pyrin—the protein involved in FMF—has a role in activating the proinflammatory cytokine interleukin (IL)-1β. We report the effectiveness of the addition of an IL-1-receptor inhibitor (anakinra) to colchicine in controlling the febrile attacks and acute phase response in an adolescent with FMF resistant to colchicine

Subclinical atherosclerosis in Behcet's disease: A systematic review and meta-analysis.

OBJECTIVE: To evaluate subclinical atherosclerosis in Behcet disease (BD), we performed a systematic review and meta-analysis of studies where atherosclerosis was determined by flow-mediated dilatation (FMD) and endothelial-mediated dilatation (EMD) and by measurement of intima media thickness (IMT) of carotid arteries. METHODS: Systematic search of EMBASE and PubMed databases from January 2000 to January 2014 according to PRISMA guidelines. RESULTS: Nine studies met the inclusion criteria on FMD/EMD, 11 on IMT and 4 on both. BD had lower FMD than controls (SMD = -0.89, 95% CI: -0.660 to -1.11, p < 0.001), which was confirmed by subgroup analyses on active and inactive patients (SMD = -1.17, 95% CI: -1.45 to -0.89 and SMD = -0.72, 95% CI: -0.97 to -0.46, p = 0.0001 for both). EMD was lower in BD but with a large estimate (SMD = 0.38, 95% CI: -0.79 to -0.03, p = 0.06, I(2) = 82.2%). IMT was greater in BD and the large estimate (SMD = 0.95, 95% CI: 0.63-1.28, p < 0.0001, I(2) = 87.6%) persisted after subgroup analysis on active and inactive patients (I(2) = 88.4% and 86.7%, respectively). Pooling IMT studies by a Newcastle Ottawa Scale of 5 and 6/7 yielded lower estimates (SMD = 0.54, 95% CI: 0.32-0.75, p < 0.0001, I(2) = 58.7% and SMD = 1.72, 95% CI: 1.35-2.09 p < 0.05, I(2) = 48.6%). CONCLUSIONS: FMD is impaired in BD even in inactive state and IMT is greater despite a degree of statistical heterogeneity that reflects the clinical heterogeneity of BD. Future prospective studies should account for risk stratification of atherosclerosis in BD

Tibial torus and toddler's fractures misdiagnosed as transient synovitis: a case series

<p>Abstract</p> <p>Introduction</p> <p>The high incidence of transient synovitis in early childhood makes it the first suspected pathology in a limping child. Trauma, which has long been regarded as a causative factor for transient synovitis, may be underestimated in a non-cooperative toddler.</p> <p>After excluding most serious conditions, such as septic arthritis, a speculative diagnosis of transient synovitis can be made, and this can easily mask a subtle musculoskeletal injury.</p> <p>Case presentations</p> <p>We report the cases of three Caucasian patients (two boys, aged 20-months- and three-years-old, and one girl, aged two-years-old), with tibial torus and toddler's fractures which were late-diagnosed due to an initial misdiagnosis of transient synovitis of the hip.</p> <p>Conclusion</p> <p>In a non-cooperative child musculoskeletal trauma can be mistaken as a simple causative factor for transient synovitis of the hip and this can easily prevent further investigation for a possible subtle musculoskeletal injury of the lower extremities.</p> <p>Our experience with the presented cases suggests the need to be more vigilant in the differential diagnosis of transient synovitis in young children.</p

Rapidly Progressive Cardiac Manifestation of Behçet's Disease Involving Conduction System and Aortic Valve

Cardiac conduction system impairment is a rare clinical manifestation of Behçet's disease. We report a patient who showed 1st degree atrioventricular block at first presentation, and showed aggravated finding of 3rd degree atrioventricular block on five months later. His cardiac manifestation finally developed to acute severe aortic regurgitation on six months later from his first cardiac manifestation. We observed this rapid progression during 6 months and successfully improved symptom and disease severity of the patient with treatment targeting Behçet's disease

Stenosis and Aneurysm of Coronary Arteries in A Patient with Behcet’s Disease

Coronary artery disease is extremely rare in patients with Behçet’s disease. We report the case of a patient with a history of Behçet’s disease who was admitted in our hospital with instable angina pectoris. The patient’s electrocardiogram was normal. Coronary angiography revealed aneurysm of the distal right coronary artery with a tight stenosis of the proximal part of the posterolateral branch. These two conditions were initially treated with immunosuppressive treatment. Three years later coronary angiography showed a total occlusion of the right coronary artery treated with medical therapy. More than fourteen cases of coronary involvement were reported in the literature but the etiopathogeny and the treatment are yet unknow

The European Renal Association - European Dialysis and Transplant Association (ERA-EDTA) Registry Annual Report 2016 : a summary

Background. This article summarizes the ERA-EDTA Registry's 2016 Annual Report, by describing the epidemiology of renal replacement therapy (RRT) for end-stage renal disease (ESRD) in 2016 within 36 countries. Methods. In 2017 and 2018, the ERA-EDTA Registry received data on patients undergoing RRT for ESRD in 2016 from 52 national or regional renal registries. In all, 32 registries provided individual patient data and 20 provided aggregated data. The incidence and prevalence of RRT and the survival probabilities of these patients were determined. Results. In 2016, the incidence of RRT for ESRD was 121 per million population (pmp), ranging from 29 pmp in Ukraine to 251 pmp in Greece. Almost two-thirds of patients were men, over half were aged >= 65 years and almost a quarter had diabetes mellitus as their primary renal diagnosis. Treatment modality at the start of RRT was haemodialysis for 84% of patients. On 31 December 2016, the prevalence of RRT was 823 pmp, ranging from 188 pmp in Ukraine to 1906 pmp in Portugal. In 2016, the transplant rate was 32 pmp, varying from 3 pmp in Ukraine to 94 pmp in the Spanish region of Catalonia. For patients commencing RRT during 2007-11, the 5-year unadjusted patient survival probability on all RRT modalities combined was 50.5%. For 2016, the incidence and prevalence of RRT were higher among men (187 and 1381 pmp) than women (101 and 827 pmp), and men had a higher rate of kidney transplantation (59 pmp) compared with women (33 pmp). For patients starting dialysis and for patients receiving a kidney transplant during 2007-11, the adjusted patient survival probabilities appeared to be higher for women than for men.Peer reviewe