Search

Introduction: Behcet's disease (BD) is classified among inflammatory vascular diseases, affecting vessels of all kinds and sizes thus affecting every tissue and organ, with unknown aetiology. Considering low frequency with which this condition occurs, variation with the signs of symptoms, lack of specific test for diagnosis, this study was done to understand the clinical behaviour of patients with BD, which will aid in diagnosing and managing patients with BD and also relapses and other associated conditions. Methodology: A hospital-based study was done in 50 adult patients of either sex, diagnosed with BD in a tertiary care hospital after obtaining ethical committee clearance during February 2020 to January 2023. The diagnosis was based on the diagnostic criteria laid down by the International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD). Results of laboratory tests, including HLA-B5, HLA-B51, and HLA-B27, were recorded. NSAIDS, steroids and immunomodulating drugs were prescribed, either individually or in combination, to treat the disease. Data analysis was done using SPSS 20. Data represented as frequency percentage and bar diagrams. Results: Majority of the patients with BD belong to age group of 41 - 60 years (84%) with mean age of the patients being 42.3±8.4. BD was seen more in males (68%). Oral apthous (94%) ulcers were the most common manifestations followed by skin lesions (52% includes erythema nodosum, pseudo folliculitis and others) and ocular involvement (50% includes anterior uveitis, posterior uveitis, pan uveitis and retinal vasculitis). Pathergy test was positive in 62% of patients. Out of 50 patients 62% had positive HLA B5, followed by HLA B51 (40%) and HLA B27 (8%). Conclusion: BD is more common in younger age group with mean age 42.3 years and in males. Most of the patients were positive for HLAB5. [ABSTRACT FROM AUTHOR]

The objectives are to describe the demographic and clinical properties of Behçet's disease (BD) and investigate their relationship with the use of biological agents. Four hundred-eighty-eight patients, (299 (61.3%) males, 189 (38.7%) females), who fulfilled the ISG classification criteria for BD were included, retrospectively. The patient's demographics, disease onset age (DOA), clinical findings of the disease, and the drugs were determined and analyzed statistically. The means of patient age and DOA were 40.7 ± 9.9 and 30.8 ± 8.8 years, respectively. The most common initial findings were oral ulcer (OU)s (30.1%), genital ulcer (GU) (27.5%), ocular involvement (OI) (12.5%), and papulopustular lesion (PPL)s (10.1%). The most common clinical manifestations were OUs (96.9%), PPLs (70.2%), HLA-B51 (64.4%), positive pathergy reaction (26.4%), GU (58.8%), OI (44.7%), erythema nodosum (29.8%), and vascular involvement (VSI) (27.3%). Although, the frequency of GU was higher in females (p = 0.01), PPLs (p = 0.001) and VSI (p = 0.001) were higher in males. Sixty-three (8.9%) patients used a biological agent. Its frequency was higher in younger patients (< 40 years) (p = 0.006), males (p = 0.012) and patients with OI (p = 0.001). Besides, the DOA (p = 0.012) and the current age (p = 0.001) were lower in biological agent users. The possibility of using biological agent was increased in males (OR = 2.2), patients with OI (OR = 2.7) and young patients (OR = 0.9). Mucocutaneous lesions are distinctive features of BD, especially OUs precede other findings. GU was more common in females and PPLs and VSI were in males. The probability of using biologics is higher in males, patients with OI, and young patients. [ABSTRACT FROM AUTHOR]

Background: Behçet's disease (BD) is a chronic, multi-systemic, recurrent condition that affects the vascular, ocular, mucocutaneous, and central nervous systems. The diagnosis of this disease depends on its clinical features, which are similar to those observed in several diseases, such as Parkinson's disease, pemphigus vulgaris, systemic lupus erythematosus, Crohn ҆s disease, and Sjӧgren's syndrome. Lysosome-mediated autophagy is a catabolic, cytoprotective mechanism that maintains cell homeostasis by degrading undesired long-lived proteins and recycling nutrients. The aim of this study was to evaluate the correlations between some autophagy-related genes (ATG5, ATG7, ATG12, LC3b, mTOR) and the pathogenesis and immunopathology of BD. The expression levels of the genes were evaluated by quantitative polymerase chain reaction (qPCR) in 101 individuals that are classified into two groups. Group 1: (n = 71) BD patients, Group 2: (n = 30) healthy controls. Results: Patients with BD had lower mRNA expression levels of ATG5 and mTOR and higher levels of LC3b mRNA than the controls. No significant differences in the levels of both ATG7 and ATG12 were observed between the two groups. According to the area under the curve analysis, LC3b was considered the best candidate biomarker among the selected markers for the diagnosis of BD. The mRNA expression of ATG5 was significantly correlated with patient age and the presence of oral ulcers. The mRNA expression of ATG7 was significantly associated with age and the presence of erythema nodosum and vascular lesions, whereas that of LC3b was significantly correlated with the presence of pustules. Conclusion: These findings indicated that elevated levels of LC3b were strongly associated with BD. Likewise, the levels of ATG5 and ATG7 were associated with the complications and outcomes of this disease. Additional assessments of the mRNA expression levels of these autophagy-related genes might prove beneficial in diagnosing this autoimmune disorder. [ABSTRACT FROM AUTHOR]

Objectives: This study aimed to determine the clinical efficacy of apremilast for oral ulcers (OUs), extra-oral manifestations, and overall disease activity in patients with Behçet's disease (BD). Methods: A systematic literature search was performed in PubMed, Embase, Cochrane Library, and Web of Science Core Collection. Studies assessing the treatment effects of apremilast in BD were included. The odds ratios (ORs) of being symptom-free for individual manifestations and mean difference (MD) of Behçet's Disease Current Activity Form (BDCAF) scores were calculated with 95% confidence intervals (CIs) at 12 and 24 weeks using a random-model meta-analysis. Results: Of 259 screened articles, eight were included. After 12 weeks of apremilast treatment the OR of symptom-free was as followings: OUs, 45.76 (95% CI, 13.23-158.31); genital ulcers, 4.56 (95% CI, 2.47-8.44); erythema nodosum, 3.59 (95% CI, 1.11-11.61); pseudofolliculitis, 2.81 95% CI, 1.29-6.15); and arthritis, 3.55 (95% CI, 1.71-7.40). Furthermore, BDCAF scores at 12 weeks were significantly reduced (MD=-1.38; -1.78 to -0.99). However, the proportion of oral-ulcer-free patients increased at 24 weeks (OR=14.88; 4.81 to 46.07). Conclusions: The currently accumulated data indicate an improvement in mucocutaneous and articular symptoms by short-term apremilast treatment in patients with BD. [ABSTRACT FROM AUTHOR]

Behçet's disease (BD) is a rare (especially in East Europe, Ukraine) systemic vasculitis of blood vessels of varying calibers throughout the body that affects various organs. The variability of the clinical features requires the involvement of doctors of different specialties in the management of such patients. The work was aimed to conduct a literature review of the intestine involvement and skin lesions in BD based on the clinical case with bloody diarrhea at the onset, and to assess the frequency of development of various clinical syndromes in intestinal BD. This is an attempt at describing a manifestation of BD with colitis and to emphasize the necessary revision of BD diagnostic criteria with special attention to early manifestations of BD with gastrointestinal tract involvement. [ABSTRACT FROM AUTHOR]

Behçet's disease (BD) is a systemic vasculitis of unknown cause. The spectrum of the disease ranges from mucocutaneous manifestations to other organ diseases with relevant morbidity. Associations between disease severity and male sex, earlier age at onset, and the presence of erythema nodosum have been described. To evaluate clinical factors associated with manifestations of severe disease in a single-center cohort. A longitudinal, prospective, unicentric cohort study with patients followed in a specialized outpatient clinic between 1981 and 2020. Severe BD was defined as a Krause total clinical severity score > 4 points. We included 243 patients, of whom 31% were male, with an average follow-up time of 14.6 years. Regarding organ manifestations, all patients had mucous manifestations (N = 243, 100%), 133 (55%) skin, 104 (43%) joint, 71 (29%) ocular, 48 (20%) vascular, 47 (19%) neurological, 22 (9%) gastrointestinal and 1 (0.4%) cardiac involvement by BD. One hundred fifty-six (64%) patients were classified as having severe BD. Severe BD was more frequent in men (OR = 2.004, p = 0.024), increasing with age (OR = 1.021 per year, p = 0.037), in the presence of skin manifestations (OR = 4.711, p < 0.001), specifically erythema nodosum (OR = 8.381, p < 0.001), and pseudofolliculitis (OR = 2.910, p < 0.001). In the multivariate model, variables independently associated with severe BD were male gender (Adjusted OR = 1.961, p = 0.047), erythema nodosum (Adjusted OR = 8.561, p < 0.001) and pseudofolliculitis (Adjusted OR = 2.372, p = 0.007). Male gender, erythema nodosum, and pseudofolliculitis were independently associated with severe BD forms and therefore should serve as warning signs to the clinician. [ABSTRACT FROM AUTHOR]

Purpose: Although it is worth to investigate seasonality in a disease that has a recurrent course, this issue has not been dealt with sufficiently for Behçet’s disease. The aim of the study was to evaluate whether or not frequencies of its mucocutaneous attacks varied between months of the year. Materials and Methods: One hundred eight patients with Behçet’s disease were retrospectively evaluated in terms of attacks of oral aphthae, genital ulcers, and erythema nodosum. For their each visit, a four-week period preceded this visit and dates of attack onsets in this period were taken into consideration. Then, by using them, mean monthly attack numbers were calculated for time series analysis. Results: Although mean monthly attack numbers were higher in May and October for oral aphthae and in August for genital ulcers, seasonalities were not verified by time series analysis. Conclusion: After a literature search on PubMed, there is only one study that detected no seasonality for mucocutaneous lesions of Behçet’s disease, and only one study detected a seasonality for oral aphthae. However, it is worth noting that time series analysis is not utilized in these studies. Since the present study is retrospective, its results should be verified by diary-based prospective studies that include time series analysis. [ABSTRACT FROM AUTHOR]

Background/aim: High mobility group box- 1 (HMGB- 1) is a nuclear protein acting as a proinflammatory molecule. The serum HMGB- 1 levels were found elevated in chronic inflammatory diseases. In this cross-sectional study, serum HMGB- 1 levels in Behcet’s disease (BD) patients and healthy controls (HC) were studied. Also, its association with disease activity scores and clinical findings were evaluated. Materials and methods: Ninety BD patients and 50 age-sex matched HC were included in the study. Disease activity scores were assessed by Behcet Disease Current Activity Form (BDCAF) and Behcet Syndrome Activity Score (BSAS). Serum HMGB- 1 levels were measured using a commercial ELISA kit. A p value of < 0.05 was considered to be statistically significant. Results: Serum HMGB- 1 levels were significantly higher in BD than in HC (43.26 pg/mL and 16.73 pg/mL; p < 0.001, respectively). Serum HMGB- 1 levels were statistically significantly associated with presence of erythema nodosum (EN) and genital ulcers in the last one month prior to recruitment (p = 0.041 and p < 0.001, respectively). BDCAF and BSAS scores were positively correlated with serum HMGB- 1 level ( p = 0.03 and p = 0.02, respectively). Conclusion: HMGB - 1 may play a role in the development of BD. Also, due to its positive correlation with disease activity indices, it can be used as a novel disease activity parameter in BD. [ABSTRACT FROM AUTHOR]

Introduction: to describe the epidemiological, clinical, therapeutic and evolving characteristics of Behçet's disease and identify prognostic factors. Methods: we have realized a retrospective, single-center study, conducted over a period of 26 years and including 130 patients presenting Behçet's disease and hospitalized in an Internal Medicine Department. Results: the mean age of the Behçet's disease at onset was 30.3 ±8.8 years and that at diagnosis was 34.6 ±9.4 years. The sex ratio (male/female) was 2.5. The mean delay of diagnosis was 53.5 months. Oral aphthosis was constant. The frequency of the manifestations was: genital aphtosis 71.5%, pseudofolliculitis 84.6%, erythema nodosum 11.5%, positive pathergy test 50%, ocular disease 36.9%, venous thrombosis 30%, arterial disease 4.6%, joint damage 30.8%, neurological disease 19.2% and digestive disease 0.8%. The male gender was significantly associated with ocular involvement (p =0.02), venous disease (p =0.01) and occurrence of relapses (p =0.01). The mean follow up was 68.5 ± 77.3 months. The poor survival prognostic factors were male gender, ocular involvement, venous disease, cardiovascular disease, a duration of follow up ≤12 months and a diagnostic delay ≤ 24 months. Conclusion: improving the prognosis of Behçet's disease requires a shortening of the time to diagnosis, multidisciplinary collaboration, intensive treatment of functional threats, regular monitoring, and patient adherence. [ABSTRACT FROM AUTHOR]

Clinically and histopathologically, the lesions raised differential diagnoses between erythema nodosum (EN), Behçet disease (BD)-associated erythema nodosum-like lesions, and polyarteritis nodosa (PAN). Dear Editor, Nodular cutaneous lesions are relatively frequently encountered in the context of Behçet disease and may present a diagnostic challenge. The utility of orcein and elastic Verhoeff-Van Gieson stain to demonstrate phlebitis in nodular cutaneous lesions of Behçet disease. [Extracted from the article]

Erythema nodosum is the most common form of panniculitis and is characterized by tender erythematous nodules mainly in the lower limbs on the pretibial area. The exact cause of erythema nodosum is unknown, although it appears to be a hypersensitivity response to a variety of antigenic stimuli. Although the etiology is mostly idiopathic, ruling out an underlying disease is imperative before diagnosing primary erythema nodosum. Erythema nodosum can be the first sign of a systemic disease that is triggered by a large group of processes, such as infections, inflammatory diseases, neoplasia, and/or drugs. The most common identifiable causes are streptococcal infections, primary tuberculosis, sarcoidosis, Behçet disease, inflammatory bowel disease, drugs, and pregnancy. We propose a diagnostic algorithm to optimize the initial work-up, hence initiating prompt and accurate management of the underlying disease. The algorithm includes an initial assessment of core symptoms, diagnostic work-up, differential diagnosis, and recommended therapies. Several treatment options for the erythema nodosum lesions have been previously reported; nevertheless, these options treat the symptoms, but not the triggering cause. Making an accurate diagnosis will allow the physician to treat the underlying cause and determine an optimal therapeutic strategy. [ABSTRACT FROM AUTHOR]

Background: The primary aim of the study is to investigate the frequency of pathergy positivity in active BD patients at the time of the first diagnosis. Its secondary aim is to compare the status of pathergy with sociodemographic, clinical and laboratory characteristics. Materials and Methods: In this study, 110 BHs were analyzed retrospectively. Sociodemographic characteristics (age, gender), clinical findings, PT status, sedimentation (mm/hr) and C-reactive protein (CRP) (mg/L) values of the patients at the time of initial diagnosis were recorded. Age of onset of disease symptoms <40 were accepted as classical onset, patients ≥40 as late-onset BD. Krause Behçet's Disease Clinical Activity Score was used for disease activity. Results: S PT was found to be positive in 36 (32.7%) of 110 patients with Behçet's disease. In PT positive patients; there was genital ulcer in 31 (86.1%), arthritis in 9(25.0%), uveitis in 7 (19.4%), deep vein thrombosis in 10 (27.8%), papulopustular lesion in 10 (27.8%), erythema nodosum in 6 (16.7%), neuropenchymal involvement in 1 (2.8%) and neurovascular involvement in 1 (2.8%). In PT negative patients, these frequencies were 66 (89.2%), 19 (25.7%), 22 (29.7%), 19 (25.7%), 12 (16.2%), 7 (9.5%), 2 (2.7%) and 1 (1.4%), respectively. The positivity of PT and the frequency of clinical findings were not statistically different (p>0.05). Median value of sedimentation (mm/h) was 16 (2-98), while the median value of CRP (mg/L) was 3.78 (0-126) in PT positive Behçet patients. In PT negative patients, these values were 17.5 (1-108) and 4.5 (0-121), respectively. When the positivity of PT and the median values of sedimentation (mm/hr) and CRP (mg/L) were compared, no significant difference was found (p>0.05). Median value of disease activity total score was 3 (1-6) in PT positive patients, while this median value was 3 (1-7) in PT negative patients. When PT positive and negative patients were compared in terms of disease activity total scores, there was no significant difference (p=0.494). While there were 34 (33.3%) patients with positive PT among classical age-onset patients, there were 2 (25.0%) among those with late onset. When classical and late-onset patients were compared in terms of PT positivity, no significant difference was found (p=1.000). Conclusions: The benefit of PT positivity in predicting the clinical severity of the disease and regulating treatment for BD is still controversial and requires further research. Perhaps new, standardized test methods that will increase the sensitivity of PT will resolve this uncertainty and make the test more valuable for clinicians. [ABSTRACT FROM AUTHOR]

Monoclonal antibody therapies have revolutionized the treatment of autoinflammatory-immune/genetic disease including spondylarthritis, asthma and rheumatoid arthritis. Behcet's disease (BD) is a multi-systemic vasculitis, which is generally recurrent aphthous lesions (RAL) as well as ocular and skin lesions. Today, the immunohistopathogenesis of BD is mostly unknown. Omalizumab (Anti-IgE humanized monoclonal antibody) therapy is given for severe persistent allergic asthma, and unintentionally it had effect on RAL. Our patient has received omalizumab treatment for 3 years. The steroid treatment was completely discontinued a month later and the systemic-steroid dependent diabetes mellitus was healed. The IL-1 β, IL-6, IL-8, IL-33, IL-25, IL-10, IL-23, and IL-17A levels were measured using an Enzyme-Linked Immunosorbent Assay (ELISA) kit. After a long-term omalizumab treatment administered, the levels of WBC, d-dimer, IL-33, IL-6, IL-25 and IL-1 β decreased. The patient's hsCRP decreased from 3 to 0.1 and Eosinophil Cationic Protein (ECP) levels decreased from 78 to 21. A significant improvement was noticed in the RAL, the asthma symptoms (cough, shortness of breath), the number of emergency admissions, and the average length of stay of the patient within the days following the initiation of the omalizumab treatment. Here, for the first time, we introduce omalizumab treatment of a patient diagnosed with BD and the examination of the treatment for the clinical manifestations and the cytokines/coagulant protein levels. A significant improvement is observed in the patient's RAL following the initiation of omalizumab. There is strong evidence that the serum proinflammatory cytokines/coagulant factors could also play an important role in the relationship between RAL and IgE-dependent vascular autoinflammation. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the changes over time in extraocular and ocular manifestations of Behçet's disease (BD) in Tunisian patients. Material and methods: Retrospective study of 246 patients divided into two groups: group 1 (147 patients examined from 1995 to 2005) and group 2 (99 patients examined from 2006 to 2017). Results: Active or scarred genital ulcers observed by physician at presentation were significantly less frequent in group 2 (47.2% vs. 29.6%; p = 0.007), as were articular involvement (50.3% vs. 34.7%; p = 0.016) and erythema nodosum (18.4% vs. 8.1%; p = 0.024). One hundred-seven patients (43.5%) developed ocular manifestations during the 23-year study period. Intermediate uveitis was significantly more frequent in group 2 than in group 1 (11.7% vs. 28.4%; p = 0.003), and posterior uveitis less frequent in group 2 than in group 1 (34.2% vs. 19.7%; p = 0.016). Patients from group 2 were more likely to have macular edema (19.8% vs. 45.6%; p = 0.001). However, better visual prognosis, with a lower rate of legal blindness, was noted in group 2. Conclusions: Changes over time included a decrease in the rate of articular involvement and cutaneous involvement. There was an increase in the rate of intermediate uveitis and a decrease in the rate of posterior uveitis over time. Despite an increase in the rate of macular edema, there was an improvement in visual prognosis, with less legal blindness over time. [ABSTRACT FROM AUTHOR]

Objective: The most important genetic factors known in the pathogenesis of Behçet's disease (BD) is HLA-B51. It has been shown that HLA-B51 positivity is high in countries on the 'Silk Road' where BD is frequent. HLA-B51 is thought to be a prognostic marker that is related to severe clinical forms of BD. In this study, we aimed to investigate the prevalence of HLA-B51 positivity in patients diagnosed with BD and the relationship between the demographic and clinical findings of the patients. Material and Methods: Between March 2013 and 2018, the files of the patients who were diagnosed with BD according to the criteria of the International Study Group for Behçet Disease in dermatology polyclinic of Selçuk University Medical Faculty were reviewed retrospectively. Forty-seven patients (22 women and 25 men) with HLA-B51 were included in the study. Results: In our study, no correlation was found between HLA-B51 positivity and gender, age at onset, family history, clinical findings (such as oral and genital ulcer, papulopustular eruption, erythema nodosum, pathergy test positivity, thrombophlebitis, ocular involvement, arthritis) and systemic manifestations. Conclusion: In previous studies, conflicting results have been reported on the relationship between HLA-B51 and BD manifestations in different ethnic groups. In our study, no statistically significant correlation was found between HLA-B51 positivity and demographic and clinical findings of Behçet patients. We believe that there is a need for larger comparative prospective studies investigating the relationship between HLA-B51 and BD in different ethnic groups. [ABSTRACT FROM AUTHOR]

Erythema nodosum leprosum (ENL) is a severe inflammatory complication of multibacillary leprosy with cutaneous and systemic involvement, usually occurring in a recurrent or chronic manner. Treatment of ENL is difficult and requires the use of systemic immunosuppressant with significant side effects, such as corticosteroids and thalidomide. [Extracted from the article]

Dear Editor, We report a rare case of erythema nodosum (EN) that responded well to tofacitinib, a Janus kinase (JAK) inhibitor, in the treatment of ulcerative colitis (UC). In fact, given the rapid EN resolution after tofacitinib treatment in our case, EN resolution preceded the intestinal symptom improvement, indicating the direct effect of tofacitinib on EN. [Extracted from the article]

Behçet's disease (BD) is a systemic vasculitis affecting prominently the veins, which is usually diagnosed in adulthood, but can occur in children younger than 16 years in about 4–26% of cases. The therapy is based on several immune-suppressive drugs; in case of inadequate control and/or complications, the biologic therapy with anti-TNF drugs has been successfully used in adults. Here, we reported one pediatric case of BD with systemic (persistent/recurrent high fever), skin and mucosal manifestations (recurrent aphthous stomatitis, anal/penile ulcers, erythema nodosum and papulo-pustules), that were unresponsive to the conventional treatment with steroids and colchicine; however, he was successfully treated with adalimumab. Compared to adult patients, the experience with adalimumab in the treatment of pediatric BD is very limited. Indeed, through a systematic search in the medical literature, we retrieved 4 case reports and 2 case series, describing BD pediatric patients treated with adalimumab, in addition to three clinical studies including some BD children. The analysis and discussion of these available clinical experiences may indicate adalimumab as an effective and safe option to treat several forms of BD, in addition to BD-related chronic uveitis. [ABSTRACT FROM AUTHOR]

Background: Patients with Behçet's disease (BD) are recognized with increased risk for venous and/or arterial thrombosis. Thrombotic tendency of BD is not known. Vascular injury, loss and dysfunction/hyperfunction of endothelial cells are believed to play a role in thrombosis development. Injury and inflammation due to vasculitis can cause platelet response with increase in mean platelet volume (MPV) and thrombosis in BD. In this study, we aimed to compare the levels of MPV between patients with BD and healthy controls, and also show its effect on thrombosis. Methods: One hundred patients with BD and 100 healthy controls were evaluated for MPV levels with clinical findings in age- gender matched case-control study. The variables of patients and controls were compared and correlated using chi-square, MannWhitney U and Spearman tests. Logistic regression analysis was used to determine independent predictors of vascular involvement and thrombosis. Results: Mean MPV was significantly higher in patients with BD than healthy controls (MPV; Patients: 9.2 ± 0.9 [7.3-12.9] vs. Controls: 8.2 ± 0.6 [6.8-10.6] fl; P < 0.0001). Platelets levels were lower than controls, but not significantly (236 ± 52.3 [112-451] vs. 245 ± 52.8 [141-467] x109/L, P = 0.55). Negative correlation was found between platelet count and MPV in patients (r = -0.51, P = 0.01). Presence of erythema nodosum (EN) and MPV were determined as predictors for vascular involvement and thrombosis (EN: P < 0.0001, OR [95% CI] = 35.4 [6.3-178.2]; MPV: P < 0.0001, OR [95% CI] = 12.8 [4.1-24.3]). Conclusion: MPV is a simple measurement for indirect monitoring of platelet activity and thrombotic potential. MPV and EN may be independent risk factors for vascular thrombosis in BD. Patients with higher MPV levels and EN in BD, might have been pursued closely for enhancing thrombosis. We advise to check the MPV and put the patients on anticoagulation if it is high. [ABSTRACT FROM AUTHOR]

Objective: To assess the efficacy of tocilizumab (TCZ) in refractory uveitis of Behcet's disease (BD). Methods: Multicentre study of patients with BD-associated uveitis. Patients were refractory to conventional and biologic immunosuppressive drugs. The main outcome measures were intraocular inflammation, macular thickness, visual acuity and corticosteroid-sparing effects. Results: We studied 11 patients (7 men) (20 affected eyes); median age 35 years. Uveitis was bilateral in nine patients. The patterns of ocular involvement were panuveitis (n = 8, with retinal vasculitis in 4), anterior uveitis (n = 2) and posterior uveitis (n = 1). Cystoid macular oedema was present in seven patients. The clinical course was recurrent (n=7) or chronic (n = 4). Before TCZ, patients had received systemic corticosteroids, conventional immunosuppressants and the following biologic agents: adalimumab (n = 8), infliximab (n=4), canakimumab (n = 1), golimumab (n = 3), etanercept (n = 1). TCZ was used as monotherapy or combined with conventional immunosuppressants at 8 mg/kg/i.v./4 weeks (n = 10) or 162mg/s.c./week (n=1). At TCZ onset the following extraocular manifestations were present: oral and/or genital ulcers (n = 7), arthritis (n = 4), folliculitis/pseudofolliculitis (n = 4), erythema nodosum (n = 2), livedo reticularis (n = 1) and neurological involvement (n = 2). TCZ yielded rapid and maintained improvement in all ocular parameters of the patients, with complete remission in eight of them. However, this was not the case for the extraocular manifestations, since TCZ was only effective in three of them. After a mean (S.D.) follow-up of 9.5 (8.05) months, TCZ was withdrawn in two cases, due to a severe infusion reaction and arthritis impairment, respectively. Conclusion: TCZ could be a therapeutic option in patients with BD and refractory uveitis. [ABSTRACT FROM AUTHOR]

Background: Erythema nodosum (EN) is the most common clinical variant of panniculitis. It may occur in association with a wide variety of causative stimuli. The aim of our study is to describe the possible etiologic factors associated with EN and compare them with the series previously reported in the literature. Materials and Method: This is a retrospective chart review of 287 patients who have presented to our clinic with tender erythematous nodular lesions and finally diagnosed as eythema nodosum between January 2010 and December 2015. Results: Our retrospective study included 239 females and 48 males (sex ratio, 5:1). Of the 287 EN patients, etiologic factor has been determined in 123 (42.85%) of the participants and this group was categorized as secondary EN. In secondary EN group the leading etiologic factor was infections (n=68, 23.69%). Other etiologic factors were Behcet's Disease (n=18, 6.27%), connective tissue disease (n=8, 2.78%), tuberculosis (n=6, 2.09%) sarcoidosis (n= 5, %1.74), drugs (n=6, 2.09%), granulomatous mastitis (n=2, 0.69%), IBD (n=2,0.69%), malignancy (n=1, 0.34%) and food supplement (n=1, 0.34%). Conclusion: Our data confirm that viral and bacterial infections are the leading causative factors of EN, followed by Behcet's Disease, pregnancy and connective tissue disease (CTD). These conditions should be investigated as part of systemic search. [ABSTRACT FROM AUTHOR]

Background: This study reports on the analysis of the application and diagnostic predictability of the revised 2014 ICBD criteria in an unselected cohort of UK patients, and the ensuing organ associations and patterns of disease.Methods: A retrospective cohort study was conducted using a database of electronic medical records. Three categories were recognised: clinically defined BD, incomplete BD and rejected diagnoses of BD. We applied the ISG 1990 and ICBD 2014 classification criteria to these subgroups to validate diagnostic accuracy against the multidisciplinary assessment.Results: Between 2012 and 2015, 281 patients underwent initial assessment at an urban tertiary care centre: 190 patients with a confirmed diagnosis of BD, 7 with an incomplete diagnosis, and 84 with a rejected diagnosis. ICBD 2014 demonstrated an estimated sensitivity of 97.89% (95% CI: 94.70 to 99.42) and positive likelihood ratio of 1.21 (1.10 to 1.28). The strongest independent predictors were: Central nervous lesions (OR = 10.57, 95% CI: 1.34 to 83.30); Genital ulceration (OR = 9.05, 95% CI: 3.35 to 24.47); Erythema nodosum (OR = 6.59, 95% CI: 2.35 to 18.51); Retinal vasculitis (OR = 6.25, 95% CI: 1.47 to 26.60); Anterior uveitis (OR = 6.16, 95% CI: 2.37 to 16.02); Posterior uveitis (OR = 4.82, 95% CI: 1.25 to 18.59).Conclusions: The ICBD 2014 criteria were more sensitive at picking up cases than ISG 1990 using the multidisciplinary assessment as the gold standard. ICBD may over-diagnose BD in a UK population. Patients who have an incomplete form of BD represent a distinct group that should not be given an early diagnostic label. Behçet's disease is a complex disease that is best diagnosed by multidisciplinary clinical assessment. Patients in the UK differ in their clinical presentation and genetic susceptibility from the original descriptions. This study also highlights an incomplete group of Behçet's patients that are less well defined by their clinical presentation. [ABSTRACT FROM AUTHOR]

Introduction: Behçet's disease (BD) is a chronic inflammatory disorder with endothelial dysfunction. Ischemiamodified albumin (IMA) is a marker used in the detection of diseases associated with oxidative stress, vascular endothelial cell dysfunction and ischemia. Mean platelet volume (MPV) signifies the platelet function and activity. Aim: To show whether MPV and IMA are useful in revealing the oxidative stress and the risk of thrombosis in patients with BD. Material and methods: Twenty-six patients with BD and 28 healthy volunteers as a control group over 18 years of age were included in the study. Serum IMA and MPV levels were analyzed in both groups. Results: The mean MPV values were identified as 0.86 ±0.15 and 0.82 ±0.08 (in the BD and control groups, respectively; p = 0.188) and the mean IMA values were 9.39 ±0.73 and 9.17 ±1.09 (in the BD and control groups, respectively; p = 0.275). There were no statistically significant differences between the groups. The IMA values of BD patients who were in the active phase were significant as compared to inactive BD patients and control groups (p = 0.041). The IMA and MPV values of the thrombotic patients, non-thrombotic patients and control groups were not significant. Conclusions: Ischemia-modified albumin may be a helpful marker of possible complications during an active period of BD [ABSTRACT FROM AUTHOR]

The article describes the case of a 20-year-old woman with a ten-year history of Behcet's disease, characterized by recurrent oral and genital ulceration, pseudofoliculitis, pathergy phenomenon, myopericarditis and occasional episodes of painful subcutaneous nodules. Topics discussed include findings on ophthalmological evaluation, a diagnosis of erythema nodosum, and improvement after four days of prednisolone.

Mouth and genital ulcers with inflamed cartilage (MAGIC) syndrome are disease that fulfilled criteria for diagnosis of Behcet's disease (BD) and relapsing polychondritis (RP). We report a 22-year-old Japanese woman presented with MAGIC syndrome and we described the clinicopathological characteristics of MAGIC syndrome based on a review of published cases from July 1985 to December 2015. In our case, the patient with oral aphthae, erythema nodosum, acne-like eruptions, uveitis, and polyarthritis fulfilled criteria for diagnosis of incomplete form of BD. The patient with uveitis, polyarthritis, and histological confirmation of chondritis also fulfilled criteria for diagnosis of RP. The patient was successfully treated with oral colchicine followed by prednisolone. The symptoms of MAGIC syndrome gradually disappeared, and the prednisolone dosage was gradually decreased and stopped. She has been in remission without active medication for a further 8 months. In the previous reports, some authors suggested that MAGIC syndrome was not a disease entity and might be RP occurring secondary to BD, another association of an autoimmune disease, or vasculitis with RP. However, the pathogenic association between MAGIC syndrome, BD, and RP is still unclear, and the number of reported cases of MAGIC syndrome is insufficient to establish a clear explanation. Therefore, further accumulation of data and careful observation of the clinical course are required to improve the understanding of MAGIC syndrome. [ABSTRACT FROM AUTHOR]

Background: Behçet’s disease (BD) is a multi-organ inflammatory disorder with mucocutaneous, ocular, neurological, musculoskeletal, gastrointestinal, and pulmonary manifestations. The aim of this study was to determine the clinical features of BD in a cohort of patients diagnosed at the University of California, Davis, United States.Methods: The medical records of 59 patients with an ICD9-code of BD were retrospectively reviewed. The International Criteria for BD was used to identify patients with a point score ≥5. The clinical manifestations of BD were investigated over a 10-year period in this cohort.Results: Twenty-six patients (20 women, six men; female:male ratio = 3.3:1.0) had a point score ≥5 based on the International Criteria. The average age of onset of BD was 29.25 ± 14.25 years. Oral ulcers (100%), genital ulcers (84.6%), articular involvement (69.2%), ocular involvement (65.3%), and skin lesions (50.0%) were the most prevalent manifestations of BD in this cohort.Conclusion: Oral ulcers were the most common presenting and lifetime sign of BD. In our cohort, oral and genital ulcers were hallmarks of BD, independent of patient demographics. Thus, patients with these signs require close surveillance for other manifestations of the disease. [ABSTRACT FROM AUTHOR]

Our goal was to determine, retrospectively, the occurrence of the symptoms of Behçet's disease in chronological order and the course of the disease. Additionally, probable factors affecting the clinical severity were investigated. A total of 368 patients (171 females and 197 males; aged 41.11 ± 10.9 years) were included in this retrospective cohort study. The chronological order of the clinical manifestations was recorded. Patients were also assessed for clinical severity score. Oral ulcer was the most common manifestation (100 %) followed by genital ulcer (89.4 %), papulopustular lesions (75 %) and articular involvement (60.1 %). Oral ulcer was the most common onset manifestation (66.8 %) followed by genital ulcer (4.9 %), erythema nodosum (3.3 %) and ocular involvement (1.4 %). The duration between the onset symptom and the fulfillment of the diagnostic criteria was 4.67 ± 5.9 years. The duration between the time point of fulfillment of diagnostic criteria and the diagnosis (2.5 ± 2.1 years) was longer in patients having only mucocutaneous lesions (2.8 ± 2.2 years) than in patients having serious organ involvements (1.9 ± 1.6 years; p < 0.01). Serious involvements such as neurological involvement and large vessel involvement had their onsets later. Mean clinical severity score was higher in male patients (5.3 ± 2.1 vs 4.8 ± 1.7; p < 0.05). In logistic regression analysis, male gender ( p = 0.03) and increased number of symptoms at diagnosis ( p < 0.001, R = 0.73) were found to be significant risk factors for severity. Mucocutaneous lesions, especially oral and/or genital ulcers, usually precede possible serious involvements; therefore, careful follow-up is mandatory. Males with increased number of organ involvements at the diagnosis are associated with more severe disease. [ABSTRACT FROM AUTHOR]

Orogenital ulcer is one of the clinical manifestations of Behçet's disease (BD). However, orogenital ulcer may be observed in various conditions, such as complex aphthous dermatitis and herpes simplex virus infections. Therefore, orogenital ulcer along with skin lesions, including acne or erythema nodosum, may be misdiagnosed as BD, but is actually pseudo-BD instead. We report here a case of pseudo-BD due to Mycobacterium tuberculosis infection in which anti-tuberculous treatment resulted in complete resolution. Furthermore, we review the literature regarding the association of BD and M. tuberculosis infection. [ABSTRACT FROM AUTHOR]

Recent genetic surveys have identified vitamin D receptor (VDR) as a susceptibility gene for several autoimmune diseases. This study was designed to investigate the association of VDR gene polymorphisms with Behçet's disease (BD) and Rheumatoid arthritis (RA). A case-control study including 151 BD, 106 RA patients and an appropriate number of healthy control subjects were performed using polymerase chain reaction (PCR) and restriction fragment length polymorphism (RFLP) techniques. Association between TaqI polymorphism and BD was marginal under codominant and recessive models (P=0.078 and P=0.058, respectively). After stratification, we found evidence for a significant association between TaqI polymorphism and BD in the elderly subjects (P=0.037). The minor ApaI a allele tended to confer an increased risk for BD susceptibility (P=0.087). BD patients with VDR homozygous AA or aa genotypes were at increased risk for development of erythema nodosum (EN) skin manifestation (P=0.038). No significant association was observed for VDR ApaI and TaqI polymorphisms with RA risk (P>0.05). TaqI and ApaI polymorphisms might be modestly implicated in BD pathogenesis. They could be considered as potential biomarkers in BD rather than susceptibility genes. However, TaqI and ApaI seemed not to be implicated in RA pathogenesis. [ABSTRACT FROM AUTHOR]

We report two cases of myelodysplastic syndrome (MDS) with trisomy 8 who had periodic fever and erythema nodosum (EN). A 74-year-old man showed periodic fever and EN. A diagnosis of MDS with trisomy 8 was made, and he was successfully treated with prednisolone (PSL). A 71-year-old man presented with intermittent fever, EN, and recurrent elevation of myogenic enzymes. Despite sustained inflammation, laboratory tests showed macrocytic anemia and thrombocytopenia. Marrow aspiration showed MDS with the chromosomal abnormality trisomy 8. He was successfully treated with PSL without repeated transient fever and elevation of creatine kinase. The results of a literature review of 35 cases of MDS with trisomy 8 and Behçet’s disease-like symptoms, such as EN, oral ulcer and intestinal ulcer, suggest that the disease entity of “trisomy 8 syndrome” may be considered, and that it is an important differential diagnosis of periodic fever and EN. [ABSTRACT FROM AUTHOR]

Recurrent oral aphthous ulcerations, genital ulcerations and cutaneous manifestations including erythema nodosum like lesions, superficial thrombophlebitis and papulopustular lesions are the most common manifestations of Behcet's disease. The pathergy reaction reflects skin hypersensitivity to trauma in Behcet's disease. Pathergy test which is characterized by the formation of a sterile pustule 24-48 h after intradermal needle prick has a high diagnostic value especially in Middle East and Mediterranean countries. Due to their high sensitivity, mucocutaneous lesions and pathergy test constitute four out of the five criteria in the International Behcet's Disease study group classification criteria. Behcet's disease has a wide clinical spectrum and since there are no specific diagnostic laboratory tests, diagnosis depends on clinical acumen of the physician. In the majority of patients with systemic findings, mucocutaneous lesions precede the onset of systemic involvement and therefore prompt recognition of mucocutaneous manifestations is of utmost importance in the early diagnosis and the outcome of the disease. [ABSTRACT FROM AUTHOR]

Background and Design: Erythema nodosum (EN) is the most common type of inflammatory nodules. Etiologic factors that lead to EN show a wide spectrum including drugs, infections, malignant and inflammatory diseases. The aim of our study was to investigate frequency of etiologic factors, clinical and laboratory findings of EN patients treated in our clinic, and to identify whether these findings were predictive for a specific etiology in these patients or not. Material and Method: A total of 72 patients diagnosed with EN in our clinic during the period 2003-2007 were included. Results: Patients were divided into two groups with regard to their etiologies. Group I was consisting of 30 cases (41.6%) in whom no underlying disease or precipitating factor were found and Group II was consisting of 42 cases (58.4%) in whom an etiologic factor was identified. Infections (n=24) were the most common identified etiologic factors followed by Behcet's disease (BD), drugs, pregnancy, and sarcoidosis. Statistical analysis revealed no difference according to age and sex characteristics, localization of disease, and the presence of fever and arthralgia as accompanying findings between the groups (p>0.05). The duration of disease was longer in Group II and patients in Group II were found to have more frequently >1 attack of EN, culture positivity and elevated ASO level when compared with that of Group I (p<0.05). However, a statistically significant difference was not found between the two groups regarding presence of leukocytosis, anemia, elevated CRP level and accelerated ESR (p>0.05). Conclusion: Infections, BD and drugs were the most frequently detected etiologic factors in our study. Our results revealed that the localization of lesions and laboratory findings like elevated ESR and CRP, leukocytosis, and anemia were not predictors of secondary EN, and that a further search for an underlying disease is necessary in patients having relapsing EN with a long disease duration. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]