Treatment Of Behcet's Disease Research Articles

Short- and long-term outcomes of surgical treatment in patients with intestinal Behcet's disease.

Behcet's disease (BD), a chronic vasculitic disorder affecting multiple organs, is characterized by recurrent oral and genital ulcers, arthritis, vasculitis, and intestinal ulcers. Although intestinal involvement of BD is common in East Asia, the efficacy and long-term outcomes of surgical treatment of intestinal BD still remain to be established. To evaluate the postoperative clinical course of intestinal BD and determine factors associated with its recurrence. Data from patients who underwent surgical treatment for intestinal BD between January 2010 and August 2021 were retrospectively reviewed. Patients' demographics, clinical features, postoperative course, complications, and follow-up data were evaluated. We analyzed 39 surgeries in 31 patients. The mean patient age was 45.1 years, and the mean interval between the diagnosis of intestinal BD and surgical treatment was 4.9 years (range 1.0-8.0 years). The most common indication for surgery was medical intractability (n = 16, 41.0%), followed by fistula or abscess (n = 11, 28.2%). Laparoscopic approaches were used in 19 patients (48.7%), and 5 patients (12.8%) underwent emergency surgeries. The most common surgical procedure was ileocecal resection (n = 18, 46.2%), followed by right colectomy (n = 11, 28.2%). A diverting stoma was created in only one patient (2.6%). During a mean follow-up period of 45 (range 8-72) months, eight cases (20.5%) of recurrence in five patients required reoperation. The interval between operations was 12.1 months (range 6.3-17.8 mo). Four patients (10.3%) experienced recurrence within 1 year postoperatively, and all eight recurrences occurred within 2 years of the initial surgery. The reoperation rates at 1 and 3 years were 10.3% and 20.5%, respectively. A redo ileocolic anastomosis was performed in all recurrent cases. In multivariate Cox regression analysis, emergency surgery [hazard ratio (HR) 9.357, 95% confidence interval (CI): 1.608-54.453, P = 0.013] and elevated C-reactive protein (CRP) levels (HR 1.154, 95%CI: 1.002-1.328, P = 0.047), but not medication use, were predictors of recurrence. Surgical resection is a feasible treatment option for complicated BD. Reoperation is associated with severe inflammatory conditions, reflected by increased CRP levels and the requirement for emergency surgery.

Surgical treatment of Behcet's disease with severe aortic regurgitation.

Behcet's disease (BD) is a multisystem inflammatory disease that is characterized by oral aphthosis, genital aphthosis, ocular lesions, and cutaneous lesions. Although BD rarely affects the cardiovascular system, its symptoms can be shown as aortic regurgitation (AR), which requires surgical intervention. Due to the special pathogenesis of BD, a low preoperative diagnosis rate and a high incidence of serious complications, such as perivalvular leakage, valve detachment, and pseudoaneurysm after prosthetic valve replacement, surgical treatment of BD with severe AR has a poor prognosis. In recent years, new surgical strategies have been developed to improve treatment efficacy for this disease. This article reviews and summarizes the evolution of surgical techniques for BD with AR and aims to provide a reference for optimizing surgical strategies, improving perioperative management, and assisting prognosis in patients suffering from BD with severe AR.

Isomer-Specific Monitoring of Sialylated N-Glycans Reveals Association of α2,3-Linked Sialic Acid Epitope With Behcet's Disease.

Behcet’s disease (BD) is an immune disease characterized by chronic and relapsing systemic vasculitis of unknown etiology, which can lead to blindness and even death. Despite continuous efforts to discover biomarkers for accurate and rapid diagnosis and optimal treatment of BD, there is still no signature marker with high sensitivity and high specificity. As the link between glycosylation and the immune system has been revealed, research on the immunological function of glycans is being actively conducted. In particular, sialic acids at the terminus of glycoconjugates are directly implicated in immune responses, cell–cell/pathogen interactions, and tumor progression. Therefore, changes in sialic acid epitope in the human body are spotlighted as a new indicator to monitor the onset and progression of immune diseases. Here, we performed global profiling of N-glycan compositions derived from the sera of 47 healthy donors and 47 BD patients using matrix-assisted laser desorption/ionization-time of flight mass spectrometry (MALDI-TOF MS) to preferentially determine BD target glycans. Then, three sialylated biantennary N-glycans were further subjected to the separation of linkage isomers and quantification using porous graphitized carbon-liquid chromatography (PGC-LC)/multiple reaction monitoring (MRM)-MS. We were able to successfully identify 11 isomers with sialic acid epitopes from the three glycan compositions consisting of Hex5HexNAc4NeuAc1, Hex5HexNAc4Fuc1NeuAc1, and Hex5HexNAc4NeuAc2. Among them, three isomers almost completely distinguished BD from control with high sensitivity and specificity with an area under the curve (AUC) of 0.945, suggesting the potential as novel BD biomarkers. In particular, it was confirmed that α2,3-sialic acid at the terminus of biantennary N-glycan was the epitope associated with BD. In this study, we present a novel approach to elucidating the association between BD and glycosylation by tracing isomeric structures containing sialic acid epitopes. Isomer-specific glycan profiling is suitable for analysis of large clinical cohorts and may facilitate the introduction of diagnostic assays for other immune diseases.

Effectiveness and Safety of Chinese Medicine Decoctions for Behcet's Disease: A Systematic Review and Meta-Analysis.

Background Behcet's disease (BD) is an autoimmune disease of systemic vasculitis with an unclear pathogenesis. Although western medicines remain the mainstay interventions, effectiveness and safety are significant challenges. Complementary and alternative medicine, including herbal medicine, are gaining more attention. Chinese medicine decoctions, which have been used for centuries, are the most common form of traditional therapies. Objective The purpose of the review was to evaluate the effectiveness and safety of Chinese medicine decoctions in the treatment of BD. Methods Randomized controlled trials (RCTs) for BD treatment with Chinese medicine decoctions were searched in six electronic databases until March 2021. Primary outcomes were total effective rate, recovery rate, and recurrence rate. Secondary outcomes were clinical feature scores (oral ulcers, eye lesions, genital ulcers, skin lesions, arthropathies, fever, and pathergy reactions) and laboratory index levels (erythrocyte sedimentation rate, C-reactive protein, and immunoglobulin A). The risk of bias was assessed with the Cochrane Handbook, and a meta-analysis was performed with RevMan 5.4.1. Results Sixteen RCTs with 924 patients were included in the review. The meta-analysis indicated that Chinese medicine decoctions were effective for BD when compared with control groups for all the primary outcomes and 7/10 of the secondary outcomes. Adverse events were reported in 11 of the 16 RCTs, with the Chinese medicine decoctions possibly having fewer adverse events than western drugs. This review included a range of classical prescriptions. An additional meta-analysis of modified Gancao Xiexin Decoction for BD treatment was conducted. Gancao Xiexin decoction is also discussed as a representative prescription, as well as high-frequency herbs, and warrants further exploration for individualized medicine and pharmacology. Conclusion Chinese medicine decoctions have the potential to be effective and safe for treating BD. However, additional well-designed RCTs are needed to confirm the findings because of the unsatisfactory quality of the included studies.

Integrative Medicine (Herbal Medicine Combined with Drug Therapy) for Behcet's Disease: A Systematic Review and Meta-Analysis of Randomized Controlled Trials.

This review aimed to investigate the efficacy of integrative medicine (herbal medicine combined with drug therapy) in the treatment of Behcet’s disease (BD). Eleven databases were searched from their inception to 7 December 2020, for randomized control trials (RCTs) that reported the effects of integrative medicine in treating BD. The risk of bias was assessed using seven domain criteria from the Cochrane Collaboration tool. Grading of Recommendations Assessment, Development, and Evaluation (GRADE) was used to assess the quality of evidence. The direction of the effect is also shown in the form of an albatross plot. Sixteen trials met the inclusion criteria and were analyzed. The overall risk of bias was determined to be uncertain. The meta-analysis showed a superior response rate with herbal medicine plus drug therapy (relative risk (RR) 1.19, 95% confidence interval (CI) 1.13 to 1.25, n = 1034, p < 0.00001, I2 = 0%, low certainty of evidence (CoE)) compared to drug therapy. Integrative medicine also lowered the recurrence rate after 2 months of follow-up (RR 0.27, 95% CI 0.09 to 0.76, n = 120, p = 0.01, I2 = 0%, low CoE). The erythrocyte sedimentation rate (ESR), C-reactive protein (CRP) level, and skin lesions were also significantly improved using integrative medicine, but equivalent effects were seen for oral ulcers, genital ulcers, and eye inflammation. Minor adverse events were reported in both groups. Our findings suggest that herbal medicine combined with drug therapy is more effective for the treatment of BD than drug therapy alone. Although the type of drug therapy used varied across the studies, integrative medicine was shown to improve the total response rate, skin lesions, the ESR, and the CRP level. However, the overall risk of bias of the studies was concerning, and the CoE was low. Information on adverse events (AEs) was also insufficient. In addition, the number of studies included for data synthesis for most outcomes was small. Future studies with rigorous RCTs may help establish the efficacy of integrative medicine in the treatment of BD.

Behcet's Disease Unmasked after Heart Transplantation

<h3>Introduction</h3> Behcet's disease (BD) is an autoinflammatory, multisystem vasculitis. Cardiac involvement is rare and clinical diagnosis is challenging. We present a case of Behcet's disease diagnosed after heart transplantation (HTx). <h3>Case Report</h3> A 24 year-old female with a history of morbid obesity, unprovoked venous thrombus and nonischemic cardiomyopathy with biventricular systolic dysfunction who presented in cardiogenic shock. Echocardiogram revealed an EF of 10-15%, LVEDD of 4.9cm, LV apical thrombus and severe RV dysfunction. Right heart catheterization showed RA 14 PA 38/23 (29) and wedge of 24 with calculated cardiac index of 1.6 and PAPi of 1.07. She required VA-ECMO and IABP followed by HM3 BiVAD as bridge to HTx. The explanted heart revealed myocyte hypertrophy and severe interstitial fibrosis, no evidence of vasculitis or inflammation. At 10 months post-transplant she presented with new painful oral and genital ulcers. Prednisone at this time was 2.5 mg daily. Complete infectious and viral work-up was negative and there was no resolution with empiric valganciclovir and antibiotics. Pathergy test was positive and treatment for BD was initiated with dapsone 150 mg twice daily and steroids. Two months, later after tapering steroids, she presented with hematochezia. Colonoscopy revealed multiple, deep, clean-based ulcers in the terminal ileum, a classic finding in BD (Figure 1). <h3>Summary</h3> BD presents a diagnostic challenge in the absence of classic clinical findings. Initial presentation is often thrombosis, which was the case in this patient years preceding her severe heart failure. Cardiac involvement is rare and to our knowledge has only been described in case reports with predominantly LV dysfunction. Long term morbidity and outcomes in BD and HTX is not well known. This is the first case reported of BD unmasked after HTx and describing biventricular failure as a possible manifestation of BD.

Antioxidant Effects of Oral Zinc Sulfate in Patients with Bechet's Disease

Treatment of Behcet disease (BD) is still considered challenging since no single agent has yet proved to reverse the underlying oxidative stress-induced inflammatory process. Zinc sulfate treatment has been shown to have an effective therapeutic outcome, however adequate exploration of the exact mechanism for its therapeutic benefit is still lacking. This study was carried out to assess and relate the oxidative stress parameters with the clinical manifestation index before and after oral zinc sulfate treatment. Twenty-five Behcet disease (BD) patients were recruited in the study besides an equal number of healthy individuals representing the control group. Patients were treated with 300mg/day oral zinc sulfate in three divided doses for one month. Blood samples were collected from the control group, patient group (before starting treatment),and treatment group (after zinc sulfate treatment) to measure serum oxidative stress parameters as well as antioxidant parameters. A dermatologist who was blind to the treatment calculated the clinical manifestation index (CMI) score and the oral ulcer parameters for each individual in the patient and treatment groups. Serum MDA, copper levels were found significantly higher in the patient group than in the control group. However, both parameters were considerably lower in the treatment group than in the patient group. Serum glutathione (GSH), zinc levels and RBC catalase activity were significantly lower in the patient group than in the control group, but they were substantially higher in the treatment group as compared to the patient group. The CMI score and oral ulcer parameters decreased significantly in the treatment group (after zinc sulfate treatment) compared to the patients’ group. The CMI demonstrated a significant positive correlation (P&lt;0.01) with the oxidative stress parameters. In conclusion, BD patients were suffering from oxidative stress, the severity of which was directly proportional to the CMI score. The antioxidant activity of oral zinc sulfate treatment i

An Overview of Conventional and Recent Treatment Options for Behcet’s Disease

Behcet’s disease (BD) is a multi-system inflammatory disorder characterized by a relapsing–remitting course. Several treatment modalities with different mechanisms of action have been evaluated for the treatment of BD. In this review, our goal is to review updated therapeutic options for BD in the light of recently published The European League Against Rheumatism (EULAR) recommendations. Behcet’s disease can be difficult to treat with conventional treatment requiring escalation of therapy. In recent years, treatment of BD has become more effective since the understanding of the pathogenesis led to the use of a wide spectrum of therapeutic agents. Data in biologics have substantially increased in BD with different organ involvements in the last decades and providing promising results, although randomized controlled trials are lacking. Behcet’s disease can affect multi-organ system therefore treatment should be determined based on the clinical presentation and organ involvement. Since there is a lack of randomized controlled trials, treatment options are usually based on expert consensus, case reports/series, and retrospective/prospective studies. As the development of biologic agents continues, it seems likely that understanding of their efficacy and safety will improve which may lead to a change in the management strategy and clinical outcomes of BD.

Efficacy and safety of metformin for Behcet's disease and its effect on Treg/Th17 balance: a single-blinded, before-after study

Behcet's disease (BD) is an autoimmune disorder that causes most commonly mouth and genital ulcerations and erythema nodules of the skin and currently has limited options of therapeutic medicines. Metformin is recently reported to suppress immune reaction, and we hypothesized that metformin could be an option for treatment of BD. Thirty patients with BD were enrolled in this perspective single-blinded, before-after study. We recorded the changes in the mucocutaneous activity index for BD (MAIBD), relapse frequency, C-reactive protein (CRP) level and erythrocyte sedimentation rate (ESR) after metformin treatment to assess the changes in the disease activity. We also analyzed the changes in the protein and mRNA expression levels of Foxp3, interleukin-35 (IL-35), transforming growth factor-β (TGF-β), Ror-γt, IL-17, and tumor necrosis factor-α (TNF-α) in these patients using ELISA and qRT-PCR. Of the 30 patients enrolled, 26 completed the trial. After the treatment, favorable responses were achieved in 88.46% (23/26) of the patients, and partial remission was obtained in 11.54% (4/26) of them. During the treatment, 8 patients complained of gastrointestinal side effects, for which 4 chose to withdraw from the study in the first week. Our results showed that metformin treatment decreased MAIBD and relapse frequency in the patients, and significantly lowered the clinical inflammatory indexes including CRP and ESR. The results of ELISA and qRT-PCR revealed that metformin treatment obviously increased Foxp3 and TGF-β expressions at both the protein and mRNA levels and significantly decreased the levels of ROR-γt, IL-17 and TNF-α as well as IL-35 level in these patients. Metformin treatment relieves the clinical symptoms, reduces the inflammatory reaction indexes and regulates the Treg/Th17 axis in patients with BD, suggesting the potential of metformin as a candidate medicine for treatment of BD.

Herbal medicine (Gancao Xiexin decoction) for Behcet disease: A systematic review protocol.

Background:Behcet disease (BD) is a systemic vasculitis that causes blood vessel inflammation throughout body and no cure exists. The purpose of this review is to evaluate the safety and efficacy of Gancao Xiexin Decoction (GCXX) in the treatment of BD and provide a clinical reference for the treatment of the refractory disease.Methods:First, the researcher will retrieve the following database based on the established search strategy: MEDLINE (PubMed), the Cochrane Central Register of Controlled Trials (CENTRAL), EMBASE, Allied and Complementary Medicine Database (AMED), China National Knowledge Infrastructure, Wanfang, VIP (Journal Integration Platform). The quality of the literature will be evaluated according to Cochrane's handbook and review inclusion criteria. Data will be extracted and data of sufficient homogeneity was combined using Review Manager (RevMan) software V5.3.5.Results:This study will provide a high quality synthesis of herbal medicine (Gancao Xiexin decoction) for Behcet disease.Conclusions:The conclusion of our systematic review will provide evidence to judge whether herbal medicine (Gancao Xiexin decoction) for Behcet disease is an effective intervention for patient with menopausal women.Trial registration number:PROSPERO CRD 42018104099.

Trisomy 8-Associated Intestinal Behcet’s Disease That Achieved Complete Remission With Mesalazine

A 39-year-old man was referred to our hospital because of ileocecal ulcer and pancytopenia. Past medical history included psoriatic arthritis and acne vulgaris. Physical examination findings showed recurrent oral ulcerations and acneiform eruptions on his body. Colonoscopy confirmed the presence of an ulcer at the ileocecal valve. Human leukocyte antigen was positive for B51. Based on the bone marrow findings of hypercellularity accompanied by trilineage dysplastic changes and cytogenetic findings of 47,XY,dup(1)(q21q32), idem+8, a definite diagnosis of intestinal Behcet’s disease (BD), incomplete type, associated with myelodysplastic syndrome (MDS) with positivity for trisomy 8 was finally confirmed. Treatment of intestinal BD with mesalazine at 3,000 mg daily induced complete ulcer healing after 4 months. Thereafter, two cycles of azacytidine was administered, followed by bone marrow transplantation. The patient has done well with continuous healing of the ulcer for a year. Differentiating episodic MDS with positivity for trisomy 8 from drug-induced MDS by examination of the bone marrow and the cytogenetic changes is indispensable. Intestinal BD patients who develop pancytopenia would probably need sufficient doses of mesalazine for treatment. J Med Cases. 2018;9(8):255-259 doi: https://doi.org/10.14740/jmc3082w

P019 CLINICAL EFFICACY OF ANTI-TNFα ANTIBODY TREATMENT IN PATIENTS WITH DEFINITE OR SUSPECTED INTESTINAL BEHCET DISEASE

Although anti-TNFα antibody treatment has been used for definite or suspected intestinal Behcet disease (BD), the efficacy of the treatment remains elusive. We performed a retrospective analysis of 10 patients with definite or suspected intestinal BD who were treated by anti-TNFα antibody treatment from July 2006 to August 2016. We evaluated the clinical response and remission rates at weeks 8, 26, and 52. We also investigated clinical predictors associated with clinical remission at week 8 and with secondary failure. The clinical activity was assessed using DAIBD (Disease Activity Index for Intestinal Behcet’s Disease). Among patients who could achieve clinical response during observation period, secondary failure of the treatment was assessed. The proportions of clinical response at weeks 8, 26, and 52 were 100%, 80% and 70%, respectively. Clinical remission rates at the respective evaluation period were 70%, 80%, and 70%. The DAIBD at baseline among 3 patients who failed to achieve clinical remission at week 8 was significantly higher than the values among the patients who could achieve clinical remission at week 8 (P = 0.01). Secondary failure was determined in 1 patient (10%) at week 26, and in 3 patients (30%) at week 52. All 3 patients who experienced secondary failure at week 52 had failed to achieve clinical remission at week 8. The remaining 7 patients who sustained clinical remission had achieved clinical remission at week 8. There was no side effect associated with anti-TNFα antibody during the average observation period of 5.5 years. Anti-TNFα antibody treatment is effective and well tolerated for definite and suspected BD. The high DAIBD value at baseline is suggested to be predictive of poor short-term and long-term clinical efficacy of the treatment in intestinal BD.

Trisomy 8-Associated Intestinal Behcet’s Disease That Achieved Complete Remission With Mesalazine

A 39-year-old man was referred to our hospital because of ileocecal ulcer and pancytopenia. Past medical history included psoriatic arthritis and acne vulgaris. Physical examination findings showed recurrent oral ulcerations and acneiform eruptions on his body. Colonoscopy confirmed the presence of an ulcer at the ileocecal valve. Human leukocyte antigen was positive for B51. Based on the bone marrow findings of hypercellularity accompanied by trilineage dysplastic changes and cytogenetic findings of 47,XY,dup(1)(q21q32), idem+8, a definite diagnosis of intestinal Behcet’s disease (BD), incomplete type, associated with myelodysplastic syndrome (MDS) with positivity for trisomy 8 was finally confirmed. Treatment of intestinal BD with mesalazine at 3,000 mg daily induced complete ulcer healing after 4 months. Thereafter, two cycles of azacytidine was administered, followed by bone marrow transplantation. The patient has done well with continuous healing of the ulcer for a year. Differentiating episodic MDS with positivity for trisomy 8 from drug-induced MDS by examination of the bone marrow and the cytogenetic changes is indispensable. Intestinal BD patients who develop pancytopenia would probably need sufficient doses of mesalazine for treatment. J Med Cases. 2018;9(8):255-259 doi: https://doi.org/10.14740/jmc3082w

Oxidative stress markers in patients with Behcet’s disease

Aim: To evaluate the role of serum malondialdehyde (MDA) and nitric oxide (NO) as oxidative biomarkers in patients with Behcet’s disease (BD) Methods: The case-control study included 44 cases and 44 controls from different areas of Duhok governorate, Iraq. All the subjects had undergone serological and hematological examinations. Results: The male to female ratio noted was 1.1:1, and the age of patients ranged between 10-63 years. The mean age of the subjects was 37 years at presentation. Serum MDA level, ESR, and total number of granulocytes were significantly higher in patients with BD in comparison to healthy controls. Furthermore, mean serum NO and the number of lymphocytes were significantly lower in BD patients compared to controls. However, total white blood cell count and average number of monocytes were comparable in both the groups. Conclusion: The study indicated the role of MDA and NO in the pathogenesis and progression of Behcet’s disease. Further research is warranted to explore its implication in diagnosis and treatment of BD.

A case of intestinal Behcet’s disease under Adalimumab

Behcet’s disease (BD) is a multisystem mucocutaneous inflammatory condition characterized by recurrent genital and oral ulcers, ocular inflammation, and can involve the gastrointestinal tract. Treatment involves the usage of immunosuppressive agents to control the disease with glucocorticoids utilized for treatment of flares. Tumor necrosis factor inhibitors are frequently used to control the disease as well. We present the case of a 40 years old African American female presenting with intestinal BD that was refractory to adalimumab therapy. In conjunction with glucocorticoids, the patient’s intestinal disease was controlled with infliximab therapy. Currently, there have been no studies comparing the efficacy of TNFα inhibitors on the treatment of BD. Future studies are needed to compare the efficacy of TNFα inhibitor agents in the treatment of intestinal manifestations of BD.

Ocular Behcet's disease is associated with aberrant methylation of interferon regulatory factor 8 (IRF8) in monocyte-derived dendritic cells.

Aberrant methylation of interferon regulatory factor 8 (IRF8) has been noted in various tumors. IRF8 has also been reported to be involved in many autoimmune diseases, including Behcet's disease (BD). However, the methylation status of IRF8 in BD has not been reported. To address this issue, we investigated whether the degree of methylation of IRF8 in dendritic cells (DCs) plays a role in the development of BD. We found a lower mRNA expression and a higher methylation level of IRF8 in active ocular BD patients as compared to normal subjects and inactive patients. Treatment with a demethylation agent, 5-Aza-2’-deoxycytidine (DAC) resulted in an increase of mRNA expression and a reduction of the IRF8 methylation level. It also down-regulated the expression of the co-stimulatory molecules CD86, CD80, CD40, and reduced the production of IL-6, IL-1β, IL-23 and IL-12. An inhibition of Th1/Th17 responses was observed as evidenced by a decreased production of IFN-γ, IL-17, and a reduction of IFN-γ/IL-17- producing CD4+ T cells following treatment with DAC. This study shows that active ocular BD patients have an aberrant IRF8 methylation status. These findings suggest that epigenetic control of IRF8 expression may offer a future target in the treatment of ocular BD.

Anti-Angiogenic Effects of Anti–Tumor Necrosis Factor α Agents in the Treatment of Behçet Disease

There are accumulating evidences on the role of dysregulated angiogenesis in Behcet disease. By considering that encouraging results are accumulating about the role of anti–tumor necrosis factor α (TNF-α) agents in the treatment of Behcet disease and that there are evidences of a role of anti-TNF-α agents in angiogenesis inhibition, it is conceivable that antiangiogenic effects of anti-TNF-α agents may contribute to the efficacy of these therapeutic arms in Behcet disease.

Long-term efficacy and safety of interferon α-2a therapy in severe refractory ophthalmic Behcet's disease.

Ophthalmic involvement is the most debilitating complication of Behcet's disease (BD). The aim of the current study is to report on the efficacy and safety of a long-term use of interferon alpha-2a (IFNα-2a) in the treatment of refractory ophthalmic BD in the Azari population of Iran. We retrospectively analyzed the clinical data of 12 patients with ophthalmic BD who were under IFNα-2a therapy. All these patients had previously been treated unsuccessfully with corticosteroid and at least one conventional immunosuppressive drug. IFNα-2a was administered at a daily dose of 6 million IU (MIU). After controlling the symptoms, a dose of 6 MIU three times per week was applied for 8-12weeks, and then, a dose of 3 MIU was administered three times per week as a subcutaneous injection. Visual acuity and total inflammatory activity index (TIAI) were used in order to assess the response to the treatment. Response to the treatment and complete eye remission were obtained in 10 (83.3%) and 7 (58.3%) patients, irrespectively. Improvement or stabilization of visual acuity was observed in 18 (81.8%) out of 22 eyes. After a mean period of 29.6months, the use of IFNa-2a was discontinued in eight (66.7%) patients. Unaltered vision for 2years after IFNa-2a discontinuation happened in eight (100%) patients. IFNa-2a is probably effective and safe in the treatment of refractory sight-threatening ophthalmic BD in the Azari population of Iran.

Cardiac manifestations in Behcet's disease.

Behcet's disease (BD) is a chronic inflammatory disorder, with vasculitis underlying the pathophysiology of its multisystemic effects. Venous pathology and thrombotic complications are hallmarks of BD. However, it has been increasingly recognised that cardiac involvement and arterial complications are also important aspects of the course of the disease. Cardiac lesions include pericarditis, endocarditis, intracardiac thrombosis, myocardial infarction, endomyocardial fibrosis, and myocardial aneurysm. Treatment of cardiovascular involvement in BD is largely empirical, and is aimed towards suppressing the vasculitis. The most challenging aspect seems to be the treatment of arterial aneurysms and thromboses due to the associated risk of bleeding. When the prognosis of cardiac involvement in BD is not good, recovery can be achieved through oral anticoagulation, immunosuppressive therapy, and colchicine use. In this review, we summarise the cardiovascular involvement, different manifestations, and treatment of BD.

Decreased expression of the aryl hydrocarbon receptor in ocular Behcet's disease.

Recent studies show that the aryl hydrocarbon receptor (AhR) is involved in immune responses. AhR is activated following interaction with its ligands, such as 6-formylindolo[3,2-b]carbazole (FICZ) and 2-(1′H-indole-3′-carbonyl)-thiazole-4-carboxylic acid methyl ester (ITE). In this study, we investigated the role of AhR activation by its endogenous ligands in the pathogenesis of ocular Behcet's disease (BD). The expression of AhR was significantly decreased in active BD patients as compared to inactive BD patients and normal controls. Both FICZ and ITE inhibited Th1 and Th17 polarization and induced the expression of IL-22 by PBMCs and by CD4+T cells in active BD patients and normal controls. Stimulation of purified CD4+T cells with FICZ or ITE caused a decreased expression of RORC, IL-17, IL-23R, and CCR6 and an increased phosphorylation of STAT3 and STAT5. The present study suggests that a decreased AhR expression is associated with disease activity in BD patients. The activation of AhR by either FICZ or ITE was able to inhibit Th1 and Th17 cell polarization. Further studies are needed to investigate whether modulation of AhR might be used in the treatment of BD.