Behcet's Syndrome

Articu lar involvement is a common manifestation of Behcet syndrome (BS), which can eventually result in significant arthralgia and necessitate total knee arthroplasty (TKA). However, outcomes of BS patients after TKA are still poorly documented in the literature. The purpose of this study was to evaluate BS as a potential risk factor for complications after TKA. BS patients undergoing primary TKA were identified from the PearlDiver Mariner database from 2010 to 2021 and compared to 10:1 matched controls. Multivariable logistic regression analyses were done for medical complications up to 90days and surgical complications up to twoyears. Ninety-day emergency department (ED) visit and inpatient readmission were also documented. A total of 4286 patients undergoing primary TKA were queried, of which 390 had BS. Patients with BS demonstrated significantly higher rates of medical complications, including deep venous thrombosis. The rates of surgical complications were similar between the two groups with the exception of periprosthetic instability, aseptic loosening, and wound complications in BS patients. Additionally, a significantly higher rate of ED visits but markedly lower rates of 90-day readmissions were noted in patients with BS. Patients with BS undergoing TKA are at higher risks of medical and surgical complications. Special considerations for a unique postoperative course with the higher complications should be made. It is crucial for orthopedic surgeons and patients alike to consider these risks when determining the expected course after TKA for patients with BS.

Henoch Schönlein purpura at diagnosis in 3 adult patients: Acute onset with peripheral nervous system dysfunction

ObjectivesSuperb microvascular imaging (SMI) provides a more sensitive assessment of small vessels compared with power and colour Doppler ultrasonography (PDUS and CDUS). We aimed to investigate the potential of SMI...

BackgroundPulmonary involvement (PI) other than pulmonary artery aneurysm (PAA) in Behcet’s syndrome (BS) is still an area of investigation.ObjectivesHerein, we aimed to retrospectively evaluate all types of pulmonary involvement associated...

Behcet's syndrome (BS) is characterized by systemic vasculitis with unknown etiology. There is evidence that more than one mechanism underlies the pathogenesis of BS, involving genetic factors and environmental stimuli. Although human leucocyte antigen-B51 is by far the most strongly associated genetic factor to BS, it accounts for less than 20% of the genetic risk, which indicates that other genetic factors remain to be discovered. Cytokines are signal molecules of the immune system which modulate the inflammatory process of nearly all immune response. The single nucleotide polymorphism (SNP) of cytokine gene have been associated with BS in many studies, involving T helper cell 1-type cytokines, interleukin 1 cytokine, interleukin 17 and chemotactic cytokine. The impact of different SNPs on BS varies, most of which affect the risk of disease, while the remaining affect the phenotype, the cumulative effect of these minor genes (SNPs) might be the genetic mechanisms of BS pathogenesis. (Chin J Ophthalmol, 2020, 56: 876-880).

In order to reduce the subjective factor in evaluating sacroiliac joint radiographs we further evaluated changes seen on standard plain films of patients suffering from Behçet's syndrome (BS) by using computed tomography (CT). Sacroiliac joint films of 20 consecutive patients with BS were mixed with those of 20 consecutive control patients and read blindly and independently by two observers. Six patients with BS met the New York criteria for sacroiliitis. Of the control patients, one had monolateral grade 1 sacroiliitis and two revealed findings consistent with osteitis condensans ilii. CT confirmed the diagnosis of sacroiliitis in patients with BS showing a high degree in at least one joint. The results of the present study suggest that the use of CT for BS patients showing sacroiliac joint changes on pelvic plain films may limit the confusion which exists about this finding in BS.

The most popular research interest in Behçet's Syndrome (BS) is directed to immunological mechanisms. However there are many ways in which BS differs from a classic autoimmune disease. The most important differences lie in the male dominance in severe disease, lack of association with other autoimmune diseases, lack of association with HLA alleles usually seen in autoimmune diseases, lack or paucity of autoantibodies and B cell hyperfunction - especially Sjögren's syndrome - and no definite T cell hypofunction in BS. Perhaps less important points are the peculiar geographic and ethnic distribution, the peculiar clinical features and the lack of response of BS to steroids. The value of immunological data on BS will much increase of we include in each experiment patients with classic autoimmune diseases along with “pure” inflammatory conditions like gout and infectious diseases.

Behçet syndrome (BS) is a multifactorial disorder with several clinical manifestations that occurs during the childbearing age. The relationship between pregnancy and BS has been previously analysed as the pregnancy immunometabolic reprogramming in case of autoinflammatory and/or autoimmune diseases can influence both the course of the disease and the pregnancy outcomes. We aim to analyse the relationship between BS and pregnancy, investigating: (1) the prevalence and clinical characteristics of disease flares during pregnancy and (2) the pregnancy outcomes in a large cohort of Italian patients with BS. This retrospective study was conducted recruiting a cohort of BS patients according to the criteria of the International Study Group for Behçet (ISG), following at the Rheumatology Department of Lucania (San Carlo Hospital, Potenza) from January 2000 to December 2021. We reviewed medical records and collected demographic and clinical data besides pregnancy-related data, in particular: maternal age, disease flares during and after pregnancy, post-partum maternal and neonatal complications (up to 6 months after pregnancy). We studied 153 pregnancies in 96/117 subjects (21/117 patients did not get pregnant). Three patients showed active disease (oral ulcers) at the time of conception. Disease flares were observed during gestation for 25/153 (16.3%) pregnancies, mainly mucocutaneous (oral and genital ulcers, erythema nodosum) and joint (arthralgia and arthritis) manifestations. A total of 27/153 (17.6%) cases of disease flares were observed after pregnancy, with mucocutaneous (oral and genital ulcers, erythema nodosum) and joint (arthralgia and arthritis) involvement, as well as anterior uveitis. Miscarriages, preterm delivery, pre-eclampsia and eclampsia were observed in 27/153 (17.6%), 19/153 (12.4%), 6/153 (3.9%) and 2/153 (1.3%) pregnancies, respectively. No cases of neonatal complications or death were observed. The results of the present study underlined that: (a) BS disease clinical symptoms do not appear to worsen during pregnancy and (b) the pregnancy does not appear to be associated with increased gestational complications and adverse maternal-fetal outcomes in case of BS. However, due to the potential adverse events, especially of the vascular involvement, a strong multidisciplinary pregnancy follow-up is recommended.

Brainstem is the most common site of involvement in neuro-Behçet syndrome (NBS). On the other hand, the critical importance of this anatomical region in the regulation of sleep has been disregarded in the literature. We aimed to investigate the microstructure of sleep in patients with Behçet syndrome (BS) and NBS. Patients were allocated to 2 groups: (1) BS without any neurological involvement and (2) NBS with brainstem lesions only. A control group was also enrolled in this study. The comparison of polysomnographic parameters between all patients (BS and NBS) with the control group showed that sleep onset was longer (p = 0.006), the duration of superficial NREM sleep stage (N2) was significantly longer (p = 0.018), and the respiratory disturbance index was significantly higher (p = 0.034) in patients. Sleep apnea and restless legs syndrome are more commonly observed in BS and NBS. Our findings emphasize the importance of questioning the quality of sleep and its disorders in patients with BS in order to better handle the common somatic complaints in these patients, such as fatigue or daytime sleepiness.

THU0299 PATIENT GLOBAL ASSESSMENT OF DISEASE ACTIVITY IN BEHÇET’S SYNDROME: A MULTICENTER STUDY

Background/Aims Neurologic involvement in Behcet’s syndrome (BS) remains an unelucidated area that needs further attention. In this study, we aimed to analyze the patterns of neurologic involvement and evaluate their association with other clinical features. Methods 800 patients with BS diagnosis according to The International Criteria for Behcet’s Disease (ICBD) followed in our center were retrospectively screened and 55 patients with central nervous system involvement and 20 patients with non-vascular BS were enrolled in this study. These patients were classified as sinus vein thrombosis (SVT), parenchymal involvement, and mixed (SVT and parenchymal). These groups were compared in terms of demographic and clinical features. Results Thirty-two (58,2%) of the patients were male. Distribution of neurologic involvement were as follows; 22 (40%) with isolated sinus vein thrombosis (SVT), 25 (%45) with isolated parenchymal, and 8 (%15) with mixed pattern. Central nervous involvement was the diagnostic feature of BS in 25 patients. Headache was the most common symptom followed by neurologic deficits and dizziness (44%, 25%, 16%). The most common sites of involvement were subcortical and periventricular areas followed by brainstem (63% vs 37%). In SVT group, positive family history of BS (p < 0.001) and previous deep vein thrombosis (DVT) (p:0.004) were found higher. Smoking history, erythema nodosum, cardiac and pulmonary involvement were significantly higher in parenchymal group (p < 0.001) (Table 1). Conclusion Although brainstem involvement is considered as more characteristic in BS, white matter and periventricular involvement were also found as common in our study. Notably, these subgroup analyses may suggest the speculation that BS shows clusters even within the neuro-vascular involvement. Disclosure R. Yıldırım: Other; Reşit Yıldırım is a member of Rheumatology journal as an associate editor. M. Dinler: None. A.E. Arslan: None. N.S.Y. Bilge: None. T. Kaşifoğlu: None.

A case of Behcet's syndrome with vascular complications is presented. This case had a familial occurrence of Behcet's syndrome. His vascular complications were deep vein thrombophlebitis of both legs, aneurysms of the left common iliac artery and the left femoral artery and the occlusion of the left subclavian artery. He was successfully operated on for the aneurysms. This paper discusses the problems accompanying the aneurysm of Behcet's syndrome. The authors pointed out that the subclavian steal syndrome may be diagnosed as neuro-Behet's syndrome and stressed the importance of vascular survay in Behcet's syndrome when the patient complaints of thrombophlebitis.

Behçet's syndrome (BS) is a rare multisystem vasculitis involving blood vessels of any size. BS aetiology is still unclear to date, and the heterogeneity of clinical expression among ethnics and genders make early diagnosis challenging. However, so far, considerable efforts have been made toward the understanding of BS, leading researchers to agree that the coexistence of some environmental triggers and a genetical susceptibility both underlie BS aetiopathogenesis. In particular, viral agents, oral microbial flora, and mucosal microbiota have been widely explored in this regard, but still no specific microorganism has been definitely linked to the disease aetiology. Likewise, the concept that some environmental factors may play a role in BS clinical presentation has emerged based on the growing evidence that disease severity is usually higher in male patients, and that diet and fatigue may be involved in disease recurrence, especially in mucocutaneous manifestations. Moreover, smoke cessation is acknowledged as a risk factor for oral ulcerations, although the underlying mechanism is still not clear. All those environmental factors play their effects through epigenetic mechanisms. The aim of this review is to discuss the evidence on the role of environmental factors in BS aetiopathogenesis and clinical course.

Behçet's syndrome (BS) is a rare chronic multi-systemic inflammatory disorder that usually involves adults between third and fourth decades of life, while pediatric and juvenile onset are relatively rare. BS young patients (YP) often develop a full-blown disease late after onset, requiring careful diagnostic workup and regular follow-up while they grow up. In this regard, the purpose of transitional programs is to ensure continuous high-quality care to YP with chronic conditions, providing them with the skills necessary to become independent and empowered adults able to chronically self-manage their disease. EULAR/PReS released the first set of standards and recommendations for transitional care (TC) of YP with juvenile-onset rheumatic diseases, but the appropriate timing for transition, the tools to evaluate patients' readiness, and indicators of transition plans effectiveness still need to be identified. Although little is known regarding TC in BS, it is easy to assume that BS YP will benefit from developmentally and disease-specifically appropriate transition plans, which may promote continuity of care, improve perceived quality of life and prevent poor disease outcomes. This perspective article discusses the key concepts and the goals of TC, addressing the potential challenges and opportunities of TC for YP with BS in clinical practice.

AB0364 DO PATIENTS PARTICIPATING IN MEETINGS REPRESENT THE ACTUAL PATIENT POPULATION IN BEHÇET SYNDROME?